#ID(s) interactor A	ID(s) interactor B	Alt. ID(s) interactor A	Alt. ID(s) interactor B	Alias(es) interactor A	Alias(es) interactor B	Interaction detection method(s)	Publication 1st author(s)	Publication Identifier(s)	Taxid interactor A	Taxid interactor B	Interaction type(s)	Source database(s)	Interaction identifier(s)	Confidence value(s)	Expansion method(s)	Biological role(s) interactor A	Biological role(s) interactor B	Experimental role(s) interactor A	Experimental role(s) interactor B	Type(s) interactor A	Type(s) interactor B	Xref(s) interactor A	Xref(s) interactor B	Interaction Xref(s)	Annotation(s) interactor A	Annotation(s) interactor B	Interaction annotation(s)	Host organism(s)	Interaction parameter(s)	Creation date	Update date	Checksum(s) interactor A	Checksum(s) interactor B	Interaction Checksum(s)	Negative	Feature(s) interactor A	Feature(s) interactor B	Stoichiometry(s) interactor A	Stoichiometry(s) interactor B	Identification method participant A	Identification method participant B
uniprotkb:Q92496-1	matrixdb:MULT_84_human	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118122|imex:IM-27449-1	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:2A, 2B|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	-	false	his tag:c-c|sufficient binding region:453-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-3	matrixdb:MULT_84_human	intact:EBI-22033638|ensembl:ENSP00000251424	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:q92496-3(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4B(isoform synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118128|imex:IM-27449-21	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000251424(transcript)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:2A, 2B, 7B|comment:weaker C3b binding to CFHR4B was observed.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:CwuvCLzcXlMtpKHDL6QP7GB4NCQ9606	-	-	false	mutation disrupting interaction strength:221-221,290-290|mutation decreasing interaction strength:221-221|his tag:c-c	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P08603-PRO_0000005894	matrixdb:MULT_84_human	intact:EBI-22114230	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118135|imex:IM-27449-41	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-1223708(chain-parent)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	chain-seq-start:19|chain-seq-end:1231	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:2A-3|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	-	false	-	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P01024-PRO_0000005915	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-6863106	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:p01024-pro_0000005915(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118145|imex:IM-27449-43	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	intact:EBI-905851(chain-parent)	-	-	chain-seq-start:1002|chain-seq-end:1303	figure legend:2C|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:zfMONKzhTZE0rk2UquV4V+6BTZI9606	rigid:xXSDkZyDo3Fe2YFPsqXaM8WXtwo	false	his tag:c-c|sufficient binding region:453-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-3	uniprotkb:P01024-PRO_0000005915	intact:EBI-22033638|ensembl:ENSP00000251424	intact:EBI-6863106	psi-mi:q92496-3(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4B(isoform synonym)	psi-mi:p01024-pro_0000005915(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118153|imex:IM-27449-23	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000251424(transcript)	intact:EBI-905851(chain-parent)	-	-	chain-seq-start:1002|chain-seq-end:1303	figure legend:2C|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:CwuvCLzcXlMtpKHDL6QP7GB4NCQ9606	rogid:zfMONKzhTZE0rk2UquV4V+6BTZI9606	rigid:P5GGFhloMYoPE8m+J9AC16++I8Y	false	his tag:c-c	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P01024	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118160|imex:IM-27449-3	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	-	-	-	figure legend:3-1|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|comment:The authors state that C3b bound to CFHR4A,  yet an anti-C3 antibody of unknown specificity was used for detection.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	rigid:g+82hntFzuyyOtbRj+U0BP0aJxs	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P00751	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-1223668|uniprotkb:Q53F89|uniprotkb:O15006|uniprotkb:Q29944|uniprotkb:Q5ST50|uniprotkb:Q96HX6|uniprotkb:Q9BTF5|uniprotkb:Q9BX92|uniprotkb:B0QZQ6|uniprotkb:Q5JP67|ensembl:ENSP00000382862|ensembl:ENSP00000388352|ensembl:ENSP00000389604|ensembl:ENSP00000391902|ensembl:ENSP00000411474|ensembl:ENSP00000411515|ensembl:ENSP00000413351|ensembl:ENSP00000414341|ensembl:ENSP00000414889|ensembl:ENSP00000416561|ensembl:ENSP00000401719	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:cfab_human(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118170|imex:IM-27449-25	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_001701.2|dip:DIP-38319N|ensembl:ENSG00000239754(gene)|ensembl:ENSG00000241253(gene)|ensembl:ENSG00000241534(gene)|ensembl:ENSG00000242335(gene)|ensembl:ENSG00000243570(gene)|ensembl:ENSG00000243649(gene)|ensembl:ENST00000399981(transcript)|ensembl:ENST00000417261(transcript)|ensembl:ENST00000419411(transcript)|ensembl:ENST00000419920(transcript)|ensembl:ENST00000424727(transcript)|ensembl:ENST00000425368(transcript)|ensembl:ENST00000426239(transcript)|ensembl:ENST00000427888(transcript)|ensembl:ENST00000433503(transcript)|ensembl:ENST00000436692(transcript)|ensembl:ENST00000455591(transcript)|go:"GO:0001848"(complement binding)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005886"(plasma membrane)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR002035(von Willebrand factor, type A)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR011360(Complement B/C2)|interpro:IPR018114|interpro:IPR028341|interpro:IPR033116|interpro:IPR035976|interpro:IPR036465|mint:P00751|rcsb pdb:1DLE|rcsb pdb:1Q0P|rcsb pdb:1RRK|rcsb pdb:1RS0|rcsb pdb:1RTK|rcsb pdb:2OK5|rcsb pdb:2WIN|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3HRZ|rcsb pdb:3HS0|rcsb pdb:6QSW|rcsb pdb:6QSX|rcsb pdb:6RAV|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6T8U|rcsb pdb:6T8V|rcsb pdb:6T8W|rcsb pdb:7JTN|rcsb pdb:7JTQ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-977606	-	-	-	figure legend:3-2|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:A6O1R+3K/DVzxKoEh2sLjEmDY/s9606	rigid:wh8GE01o85x37KU/TNTpuAOB5x8	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P27918	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-9038570|uniprotkb:O15134|uniprotkb:O15135|uniprotkb:O15136|uniprotkb:O75826|ensembl:ENSP00000247153|ensembl:ENSP00000380189	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:prop_human(display_long)|uniprotkb:CFP(gene name)|psi-mi:CFP(display_short)|uniprotkb:PFC(gene name synonym)|uniprotkb:Complement factor P(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118180|imex:IM-27449-5	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_001138724.1|refseq:NP_002612.1|ensembl:ENSG00000126759(gene)|ensembl:ENST00000247153(transcript)|ensembl:ENST00000396992(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0006955"(immune response)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0035580"(specific granule lumen)|go:"GO:0042742"(defense response to bacterium)|go:"GO:1904724"(tertiary granule lumen)|interpro:IPR000884(Thrombospondin, type I)|interpro:IPR036383|rcsb pdb:1W0R|rcsb pdb:1W0S|rcsb pdb:6RUR|rcsb pdb:6RUS|rcsb pdb:6RUV|rcsb pdb:6RV6|rcsb pdb:6S08|rcsb pdb:6S0A|rcsb pdb:6S0B|rcsb pdb:6SEJ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-5083635|reactome:R-HSA-5173214|reactome:R-HSA-6798695|reactome:R-HSA-977606	-	-	-	figure legend:3-3|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:XBExMxbnkfjvnV9zHMRZgxZWKaw9606	rigid:PPqkK5Hm+PviVGNNgQGd6dBTZWg	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-3	uniprotkb:P01024	intact:EBI-22033638|ensembl:ENSP00000251424	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:q92496-3(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4B(isoform synonym)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118190|imex:IM-27449-27	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000251424(transcript)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	-	-	-	figure legend:3-4, 7C|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|comment:The authors state that C3b bound to CFHR4A,  yet an anti-C3 antibody of unknown specificity was used for detection.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:CwuvCLzcXlMtpKHDL6QP7GB4NCQ9606	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	rigid:l0sQJanhUPXicS37RpKlr190btg	false	his tag:c-c|mutation decreasing interaction strength:221-221|mutation disrupting interaction strength:221-221,290-290	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-3	uniprotkb:P00751	intact:EBI-22033638|ensembl:ENSP00000251424	intact:EBI-1223668|uniprotkb:Q53F89|uniprotkb:O15006|uniprotkb:Q29944|uniprotkb:Q5ST50|uniprotkb:Q96HX6|uniprotkb:Q9BTF5|uniprotkb:Q9BX92|uniprotkb:B0QZQ6|uniprotkb:Q5JP67|ensembl:ENSP00000382862|ensembl:ENSP00000388352|ensembl:ENSP00000389604|ensembl:ENSP00000391902|ensembl:ENSP00000411474|ensembl:ENSP00000411515|ensembl:ENSP00000413351|ensembl:ENSP00000414341|ensembl:ENSP00000414889|ensembl:ENSP00000416561|ensembl:ENSP00000401719	psi-mi:q92496-3(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4B(isoform synonym)	psi-mi:cfab_human(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118200|imex:IM-27449-45	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000251424(transcript)	refseq:NP_001701.2|dip:DIP-38319N|ensembl:ENSG00000239754(gene)|ensembl:ENSG00000241253(gene)|ensembl:ENSG00000241534(gene)|ensembl:ENSG00000242335(gene)|ensembl:ENSG00000243570(gene)|ensembl:ENSG00000243649(gene)|ensembl:ENST00000399981(transcript)|ensembl:ENST00000417261(transcript)|ensembl:ENST00000419411(transcript)|ensembl:ENST00000419920(transcript)|ensembl:ENST00000424727(transcript)|ensembl:ENST00000425368(transcript)|ensembl:ENST00000426239(transcript)|ensembl:ENST00000427888(transcript)|ensembl:ENST00000433503(transcript)|ensembl:ENST00000436692(transcript)|ensembl:ENST00000455591(transcript)|go:"GO:0001848"(complement binding)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005886"(plasma membrane)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR002035(von Willebrand factor, type A)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR011360(Complement B/C2)|interpro:IPR018114|interpro:IPR028341|interpro:IPR033116|interpro:IPR035976|interpro:IPR036465|mint:P00751|rcsb pdb:1DLE|rcsb pdb:1Q0P|rcsb pdb:1RRK|rcsb pdb:1RS0|rcsb pdb:1RTK|rcsb pdb:2OK5|rcsb pdb:2WIN|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3HRZ|rcsb pdb:3HS0|rcsb pdb:6QSW|rcsb pdb:6QSX|rcsb pdb:6RAV|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6T8U|rcsb pdb:6T8V|rcsb pdb:6T8W|rcsb pdb:7JTN|rcsb pdb:7JTQ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-977606	-	-	-	figure legend:3-5, 7C|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:CwuvCLzcXlMtpKHDL6QP7GB4NCQ9606	rogid:A6O1R+3K/DVzxKoEh2sLjEmDY/s9606	rigid:SFpHHYDr5O/zr65aZG9jpjE14FI	false	his tag:c-c|mutation decreasing interaction strength:221-221|mutation disrupting interaction strength:221-221,290-290	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-3	uniprotkb:P27918	intact:EBI-22033638|ensembl:ENSP00000251424	intact:EBI-9038570|uniprotkb:O15134|uniprotkb:O15135|uniprotkb:O15136|uniprotkb:O75826|ensembl:ENSP00000247153|ensembl:ENSP00000380189	psi-mi:q92496-3(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4B(isoform synonym)	psi-mi:prop_human(display_long)|uniprotkb:CFP(gene name)|psi-mi:CFP(display_short)|uniprotkb:PFC(gene name synonym)|uniprotkb:Complement factor P(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118208|imex:IM-27449-7	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000251424(transcript)	refseq:NP_001138724.1|refseq:NP_002612.1|ensembl:ENSG00000126759(gene)|ensembl:ENST00000247153(transcript)|ensembl:ENST00000396992(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0006955"(immune response)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0035580"(specific granule lumen)|go:"GO:0042742"(defense response to bacterium)|go:"GO:1904724"(tertiary granule lumen)|interpro:IPR000884(Thrombospondin, type I)|interpro:IPR036383|rcsb pdb:1W0R|rcsb pdb:1W0S|rcsb pdb:6RUR|rcsb pdb:6RUS|rcsb pdb:6RUV|rcsb pdb:6RV6|rcsb pdb:6S08|rcsb pdb:6S0A|rcsb pdb:6S0B|rcsb pdb:6SEJ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-5083635|reactome:R-HSA-5173214|reactome:R-HSA-6798695|reactome:R-HSA-977606	-	-	-	figure legend:3-6, 7C|comment:Unpurified Human serum, diluted in Mg-EGTA buffer was used, to allow activation of AP pathway only.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:CwuvCLzcXlMtpKHDL6QP7GB4NCQ9606	rogid:XBExMxbnkfjvnV9zHMRZgxZWKaw9606	rigid:ul2PDENY98rHfrwTxT0ZeIJB04s	false	his tag:c-c|mutation decreasing interaction strength:221-221|mutation disrupting interaction strength:221-221,290-290	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P00746	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-1223696|uniprotkb:Q5U5S1|uniprotkb:Q86VJ5|uniprotkb:Q8N4E0|uniprotkb:Q8WZB4|uniprotkb:B4DV76|ensembl:ENSP00000332139|ensembl:ENSP00000478745	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:cfad_human(display_long)|uniprotkb:CFD(gene name)|psi-mi:CFD(display_short)|uniprotkb:DF(gene name synonym)|uniprotkb:PFD(gene name synonym)|uniprotkb:C3 convertase activator(gene name synonym)|uniprotkb:Properdin factor D(gene name synonym)|uniprotkb:Adipsin(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118216|imex:IM-27449-9	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_001919.2|refseq:NP_001304264.1|ensembl:ENSG00000197766(gene)|ensembl:ENSG00000274619(gene)|ensembl:ENST00000327726(transcript)|ensembl:ENST00000617994(transcript)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0006508"(proteolysis)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0007219"(Notch signaling pathway)|go:"GO:0008236"(serine-type peptidase activity)|go:"GO:0009617"(response to bacterium)|go:"GO:0031093"(platelet alpha granule lumen)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0070062"(extracellular exosome)|go:"GO:1904813"(ficolin-1-rich granule lumen)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR018114|interpro:IPR033116|interpro:IPR037561|interpro:IPR043504|rcsb pdb:1BIO|rcsb pdb:1DFP|rcsb pdb:1DIC|rcsb pdb:1DST|rcsb pdb:1DSU|rcsb pdb:1FDP|rcsb pdb:1HFD|rcsb pdb:2XW9|rcsb pdb:2XWA|rcsb pdb:2XWB|rcsb pdb:4CBN|rcsb pdb:4CBO|rcsb pdb:4D9R|rcsb pdb:5FBE|rcsb pdb:5FBI|rcsb pdb:5FCK|rcsb pdb:5MT0|rcsb pdb:5MT4|rcsb pdb:5NAR|rcsb pdb:5NAT|rcsb pdb:5NAW|rcsb pdb:5NB6|rcsb pdb:5NB7|rcsb pdb:5NBA|rcsb pdb:5TCA|rcsb pdb:5TCC|rcsb pdb:6FTY|rcsb pdb:6FTZ|rcsb pdb:6FUG|rcsb pdb:6FUH|rcsb pdb:6FUI|rcsb pdb:6FUJ|rcsb pdb:6FUT|rcsb pdb:6QMR|rcsb pdb:6QMT|rcsb pdb:6VMJ|rcsb pdb:6VMK|reactome:R-HSA-114608|reactome:R-HSA-173736|reactome:R-HSA-6798695	-	-	-	figure legend:4A|comment:CFHR4A  served as a platform enabling the formation of an active C3 convertase -CFHR4A-C3bBb.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:G9Jd0KXMDGx6ds9rkRNVrrzrgtw9606	-	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P27918	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-9038570|uniprotkb:O15134|uniprotkb:O15135|uniprotkb:O15136|uniprotkb:O75826|ensembl:ENSP00000247153|ensembl:ENSP00000380189	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:prop_human(display_long)|uniprotkb:CFP(gene name)|psi-mi:CFP(display_short)|uniprotkb:PFC(gene name synonym)|uniprotkb:Complement factor P(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118216|imex:IM-27449-9	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_001138724.1|refseq:NP_002612.1|ensembl:ENSG00000126759(gene)|ensembl:ENST00000247153(transcript)|ensembl:ENST00000396992(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0006955"(immune response)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0035580"(specific granule lumen)|go:"GO:0042742"(defense response to bacterium)|go:"GO:1904724"(tertiary granule lumen)|interpro:IPR000884(Thrombospondin, type I)|interpro:IPR036383|rcsb pdb:1W0R|rcsb pdb:1W0S|rcsb pdb:6RUR|rcsb pdb:6RUS|rcsb pdb:6RUV|rcsb pdb:6RV6|rcsb pdb:6S08|rcsb pdb:6S0A|rcsb pdb:6S0B|rcsb pdb:6SEJ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-5083635|reactome:R-HSA-5173214|reactome:R-HSA-6798695|reactome:R-HSA-977606	-	-	-	figure legend:4A|comment:CFHR4A  served as a platform enabling the formation of an active C3 convertase -CFHR4A-C3bBb.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:XBExMxbnkfjvnV9zHMRZgxZWKaw9606	-	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	matrixdb:MULT_84_human	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118216|imex:IM-27449-9	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:4A|comment:CFHR4A  served as a platform enabling the formation of an active C3 convertase -CFHR4A-C3bBb.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	-	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P00751	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-1223668|uniprotkb:Q53F89|uniprotkb:O15006|uniprotkb:Q29944|uniprotkb:Q5ST50|uniprotkb:Q96HX6|uniprotkb:Q9BTF5|uniprotkb:Q9BX92|uniprotkb:B0QZQ6|uniprotkb:Q5JP67|ensembl:ENSP00000382862|ensembl:ENSP00000388352|ensembl:ENSP00000389604|ensembl:ENSP00000391902|ensembl:ENSP00000411474|ensembl:ENSP00000411515|ensembl:ENSP00000413351|ensembl:ENSP00000414341|ensembl:ENSP00000414889|ensembl:ENSP00000416561|ensembl:ENSP00000401719	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:cfab_human(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118216|imex:IM-27449-9	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	refseq:NP_001701.2|dip:DIP-38319N|ensembl:ENSG00000239754(gene)|ensembl:ENSG00000241253(gene)|ensembl:ENSG00000241534(gene)|ensembl:ENSG00000242335(gene)|ensembl:ENSG00000243570(gene)|ensembl:ENSG00000243649(gene)|ensembl:ENST00000399981(transcript)|ensembl:ENST00000417261(transcript)|ensembl:ENST00000419411(transcript)|ensembl:ENST00000419920(transcript)|ensembl:ENST00000424727(transcript)|ensembl:ENST00000425368(transcript)|ensembl:ENST00000426239(transcript)|ensembl:ENST00000427888(transcript)|ensembl:ENST00000433503(transcript)|ensembl:ENST00000436692(transcript)|ensembl:ENST00000455591(transcript)|go:"GO:0001848"(complement binding)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005886"(plasma membrane)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR002035(von Willebrand factor, type A)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR011360(Complement B/C2)|interpro:IPR018114|interpro:IPR028341|interpro:IPR033116|interpro:IPR035976|interpro:IPR036465|mint:P00751|rcsb pdb:1DLE|rcsb pdb:1Q0P|rcsb pdb:1RRK|rcsb pdb:1RS0|rcsb pdb:1RTK|rcsb pdb:2OK5|rcsb pdb:2WIN|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3HRZ|rcsb pdb:3HS0|rcsb pdb:6QSW|rcsb pdb:6QSX|rcsb pdb:6RAV|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6T8U|rcsb pdb:6T8V|rcsb pdb:6T8W|rcsb pdb:7JTN|rcsb pdb:7JTQ|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-977606	-	-	-	figure legend:4A|comment:CFHR4A  served as a platform enabling the formation of an active C3 convertase -CFHR4A-C3bBb.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:A6O1R+3K/DVzxKoEh2sLjEmDY/s9606	-	false	his tag:c-c|sufficient binding region:206-578	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P01024-PRO_0000005908	uniprotkb:Q92496-1	intact:EBI-6863145	intact:EBI-22033617|ensembl:ENSP00000477162	psi-mi:p01024-pro_0000005908(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118340|imex:IM-27449-17	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	-	chain-seq-start:23|chain-seq-end:667	-	figure legend:Supp. Fig 1B|comment:"Weak binding.  (Merck):-  The iC3b fragment is a glycoprotein composed of two C3alpha-prime polypeptides of M.W. 43,000 and M.W. 63,000 which are disulfide bonded to the intact C3 beta-chain ."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:T/k1bkPGxCoHGYHH0IP28dPxaNo9606	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rigid:Ul45BHG2GkcRvKxKZVmvMITYZPY	false	disulfide crosslinked residues:?-?	-	1	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P01024-PRO_0000005908	uniprotkb:P01024-PRO_0000005911	intact:EBI-6863145	intact:EBI-12735725	psi-mi:p01024-pro_0000005908(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118340|imex:IM-27449-17	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-905851(chain-parent)	-	chain-seq-start:23|chain-seq-end:667	chain-seq-start:749|chain-seq-end:1663	figure legend:Supp. Fig 1B|comment:"Weak binding.  (Merck):-  The iC3b fragment is a glycoprotein composed of two C3alpha-prime polypeptides of M.W. 43,000 and M.W. 63,000 which are disulfide bonded to the intact C3 beta-chain ."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:T/k1bkPGxCoHGYHH0IP28dPxaNo9606	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rigid:Ul45BHG2GkcRvKxKZVmvMITYZPY	false	disulfide crosslinked residues:?-?	disulfide crosslinked residues:?-?|sufficient binding region:207..207-915..915	1	2	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P01024-PRO_0000005908	uniprotkb:P08603-PRO_0000005894	intact:EBI-6863145	intact:EBI-22114230	psi-mi:p01024-pro_0000005908(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118351|imex:IM-27449-33	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-1223708(chain-parent)	-	chain-seq-start:23|chain-seq-end:667	chain-seq-start:19|chain-seq-end:1231	figure legend:Supp. Fig 1B|comment:"(Merck):- The iC3b fragment is a glycoprotein composed of two C3alpha-prime polypeptides of M.W. 43,000 and M.W. 63,000 which are disulfide bonded to the intact C3 beta-chain ."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:T/k1bkPGxCoHGYHH0IP28dPxaNo9606	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	rigid:rDtY6+Qyibc43A4XwarzerMtVrg	false	disulfide crosslinked residues:?-?	-	1	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P01024-PRO_0000005908	uniprotkb:P01024-PRO_0000005911	intact:EBI-6863145	intact:EBI-12735725	psi-mi:p01024-pro_0000005908(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118351|imex:IM-27449-33	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-905851(chain-parent)	-	chain-seq-start:23|chain-seq-end:667	chain-seq-start:749|chain-seq-end:1663	figure legend:Supp. Fig 1B|comment:"(Merck):- The iC3b fragment is a glycoprotein composed of two C3alpha-prime polypeptides of M.W. 43,000 and M.W. 63,000 which are disulfide bonded to the intact C3 beta-chain ."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/28	rogid:T/k1bkPGxCoHGYHH0IP28dPxaNo9606	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rigid:rDtY6+Qyibc43A4XwarzerMtVrg	false	disulfide crosslinked residues:?-?	disulfide crosslinked residues:?-?|sufficient binding region:207..207-915..915	1	2	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
matrixdb:MULT_84_human	uniprotkb:P08603-PRO_0000005894	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-22114230	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118361|imex:IM-27449-35	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223708(chain-parent)	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:19|chain-seq-end:1231	figure legend:Supp. Fig 1B|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	false	-	-	1	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	matrixdb:MULT_84_human	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0407"(direct interaction)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118370|imex:IM-27449-19	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:Supp. Fig 1B|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	-	false	-	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
matrixdb:MULT_84_human	uniprotkb:P01024	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118257|imex:IM-27449-11	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:4B|comment:"The C3a fragment generated by the C3 convertase (C3bBb) was detected."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P00751-PRO_0000027547	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-21988629	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118257|imex:IM-27449-11	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223668(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:260|chain-seq-end:764	figure legend:4B|comment:"The C3a fragment generated by the C3 convertase (C3bBb) was detected."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P01024	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118263|imex:IM-27449-13	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:4B|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P00751-PRO_0000027547	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-21988629	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118263|imex:IM-27449-13	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223668(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:260|chain-seq-end:764	figure legend:4B|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:Q92496-1	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-22033617|ensembl:ENSP00000477162	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118263|imex:IM-27449-13	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:4B|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P01024	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118291|imex:IM-27449-31	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:5C|comment:"The C3a fragment generated by the C3 convertase (C3bBb) was detected."|comment:Generation of C3a from C3 by the C3bBb convertase was strongly reduced even at low FH concentrations.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P00751-PRO_0000027547	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-21988629	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118291|imex:IM-27449-31	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223668(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:260|chain-seq-end:764	figure legend:5C|comment:"The C3a fragment generated by the C3 convertase (C3bBb) was detected."|comment:Generation of C3a from C3 by the C3bBb convertase was strongly reduced even at low FH concentrations.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P08603-PRO_0000005894	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-22114230	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118291|imex:IM-27449-31	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0586"(inhibitor)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223708(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:19|chain-seq-end:1231	figure legend:5C|comment:"The C3a fragment generated by the C3 convertase (C3bBb) was detected."|comment:Generation of C3a from C3 by the C3bBb convertase was strongly reduced even at low FH concentrations.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P01024	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-905851|uniprotkb:A7E236|ensembl:ENSP00000245907	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:co3_human(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118298|imex:IM-27449-15	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	refseq:NP_000055.2|dip:DIP-35180N|ensembl:ENSG00000125730(gene)|ensembl:ENST00000245907(transcript)|go:"GO:0001798"(positive regulation of type IIa hypersensitivity)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0001970"(positive regulation of activation of membrane attack complex)|go:"GO:0004866"(endopeptidase inhibitor activity)|go:"GO:0005102"(signaling receptor binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0005788"(endoplasmic reticulum lumen)|go:"GO:0005886"(plasma membrane)|go:"GO:0006631"(fatty acid metabolic process)|go:"GO:0006954"(inflammatory response)|go:"GO:0006955"(immune response)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0007165"(signal transduction)|go:"GO:0007186"(G protein-coupled receptor signaling pathway)|go:"GO:0009617"(response to bacterium)|go:"GO:0009986"(cell surface)|go:"GO:0010575"(positive regulation of vascular endothelial growth factor production)|go:"GO:0010828"(positive regulation of glucose transmembrane transport)|go:"GO:0010866"(regulation of triglyceride biosynthetic process)|go:"GO:0010884"(positive regulation of lipid storage)|go:"GO:0016322"(neuron remodeling)|go:"GO:0031715"(C5L2 anaphylatoxin chemotactic receptor binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0034774"(secretory granule lumen)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0035846"(oviduct epithelium development)|go:"GO:0045745"(positive regulation of G protein-coupled receptor signaling pathway)|go:"GO:0045766"(positive regulation of angiogenesis)|go:"GO:0048260"(positive regulation of receptor-mediated endocytosis)|go:"GO:0060100"(positive regulation of phagocytosis, engulfment)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:0097242"(amyloid-beta clearance)|go:"GO:0097278"(complement-dependent cytotoxicity)|go:"GO:0150062"(complement-mediated synapse pruning)|go:"GO:0150064"(vertebrate eye-specific patterning)|go:"GO:1905114"(cell surface receptor signaling pathway involved in cell-cell signaling)|go:"GO:2000427"(positive regulation of apoptotic cell clearance)|interpro:IPR000020(Anaphylatoxin/fibulin)|interpro:IPR001134(Netrin, C-terminal)|interpro:IPR001599(Alpha-2-macroglobulin)|interpro:IPR001840(Complement C3a/C4a/C5a anaphylatoxin)|interpro:IPR002890(Alpha-2-macroglobulin, N-terminal)|interpro:IPR008930(Terpenoid cylases/protein prenyltransferase alpha-alpha toroid)|interpro:IPR008993(TIMP-like, OB-fold)|interpro:IPR009048(Alpha-macroglobulin, receptor-binding)|interpro:IPR011625(Alpha-2-macroglobulin, N-terminal 2)|interpro:IPR011626(A-macroglobulin complement component)|interpro:IPR013783(Immunoglobulin-like fold)|interpro:IPR018081|interpro:IPR018933|interpro:IPR019742|interpro:IPR035711|interpro:IPR035815|interpro:IPR036595|interpro:IPR040839|interpro:IPR041425|interpro:IPR041555|mint:P01024|rcsb pdb:1C3D|rcsb pdb:1GHQ|rcsb pdb:1W2S|rcsb pdb:2A73|rcsb pdb:2A74|rcsb pdb:2GOX|rcsb pdb:2I07|rcsb pdb:2ICE|rcsb pdb:2ICF|rcsb pdb:2NOJ|rcsb pdb:2QKI|rcsb pdb:2WII|rcsb pdb:2WIN|rcsb pdb:2WY7|rcsb pdb:2WY8|rcsb pdb:2XQW|rcsb pdb:2XWB|rcsb pdb:2XWJ|rcsb pdb:3D5R|rcsb pdb:3D5S|rcsb pdb:3G6J|rcsb pdb:3L3O|rcsb pdb:3L5N|rcsb pdb:3NMS|rcsb pdb:3OED|rcsb pdb:3OHX|rcsb pdb:3OXU|rcsb pdb:3RJ3|rcsb pdb:3T4A|rcsb pdb:4HW5|rcsb pdb:4HWJ|rcsb pdb:4I6O|rcsb pdb:4M76|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5FO7|rcsb pdb:5FO8|rcsb pdb:5FO9|rcsb pdb:5FOA|rcsb pdb:5FOB|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:6EHG|rcsb pdb:6RMT|rcsb pdb:6RMU|rcsb pdb:6RUR|rcsb pdb:6RUV|rcsb pdb:6S0B|rcsb pdb:6YO6|reactome:R-HSA-173736|reactome:R-HSA-174577|reactome:R-HSA-198933|reactome:R-HSA-375276|reactome:R-HSA-381426|reactome:R-HSA-418594|reactome:R-HSA-6798695|reactome:R-HSA-8957275|reactome:R-HSA-9660826|reactome:R-HSA-977606	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:5C|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|comment:The decay of the CFHR4A-C3bBb convertase is less efficiently accelerated by FH as compared with that of the C3bBb convertase.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:6Axa89cCPpUXFsLnd7sCurZzLYI9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P00751-PRO_0000027547	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-21988629	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118298|imex:IM-27449-15	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223668(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:260|chain-seq-end:764	figure legend:5C|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|comment:The decay of the CFHR4A-C3bBb convertase is less efficiently accelerated by FH as compared with that of the C3bBb convertase.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:P08603-PRO_0000005894	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-22114230	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118298|imex:IM-27449-15	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0586"(inhibitor)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-1223708(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	chain-seq-start:19|chain-seq-end:1231	figure legend:5C|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|comment:The decay of the CFHR4A-C3bBb convertase is less efficiently accelerated by FH as compared with that of the C3bBb convertase.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
matrixdb:MULT_84_human	uniprotkb:Q92496-1	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	intact:EBI-22033617|ensembl:ENSP00000477162	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118298|imex:IM-27449-15	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:1302"(stable complex)	psi-mi:"MI:0326"(protein)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	go:"GO:0004252"(serine-type endopeptidase activity)	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	-	figure legend:5C|comment:The CFHR4A–bound convertase was active as demonstrated by the conversion of C3 to C3a .|comment:The decay of the CFHR4A-C3bBb convertase is less efficiently accelerated by FH as compared with that of the C3bBb convertase.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	-	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	false	-	-	-	-	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)
uniprotkb:P01024-PRO_0000005911	uniprotkb:P05156-PRO_0000027568	intact:EBI-12735725	intact:EBI-11786583	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p05156-pro_0000027568(display_long)|uniprotkb:CFI(gene name)|psi-mi:CFI(display_short)|uniprotkb:IF(gene name synonym)|uniprotkb:C3B/C4B inactivator(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118435|imex:IM-27449-37	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-9352022(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	chain-seq-start:749|chain-seq-end:1663	chain-seq-start:19|chain-seq-end:583	figure legend:Supp. fig 2A,  lane 2|comment:Factor I cleaves the a'  chain of the C3b complex.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rogid:A0swF+P13pIz6IYOWBbpRNi+l+49606	rigid:thUQKdqUzNgp2fkLhfwJFY473uc	false	-	-	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P01024-PRO_0000005911	uniprotkb:P08603-PRO_0000005894	intact:EBI-12735725	intact:EBI-22114230	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118435|imex:IM-27449-37	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-1223708(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	chain-seq-start:749|chain-seq-end:1663	chain-seq-start:19|chain-seq-end:1231	figure legend:Supp. fig 2A,  lane 2|comment:Factor I cleaves the a'  chain of the C3b complex.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	rigid:thUQKdqUzNgp2fkLhfwJFY473uc	false	-	-	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P01024-PRO_0000005911	uniprotkb:P05156-PRO_0000027568	intact:EBI-12735725	intact:EBI-11786583	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p05156-pro_0000027568(display_long)|uniprotkb:CFI(gene name)|psi-mi:CFI(display_short)|uniprotkb:IF(gene name synonym)|uniprotkb:C3B/C4B inactivator(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118441|imex:IM-27449-39	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-9352022(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	chain-seq-start:749|chain-seq-end:1663	chain-seq-start:19|chain-seq-end:583	figure legend:Supp. fig 2A,  lane 3|comment:Factor I cleaves the a'  chain of the C3b complex.|comment:CFHR4A does not display cofactor activity, it enhances the cofactor activity of FH.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rogid:A0swF+P13pIz6IYOWBbpRNi+l+49606	rigid:JXnXHHpruX8l90QavbpphKaXO0I	false	-	-	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P01024-PRO_0000005911	uniprotkb:P08603-PRO_0000005894	intact:EBI-12735725	intact:EBI-22114230	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118441|imex:IM-27449-39	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0684"(ancillary)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-1223708(chain-parent)	go:"GO:0004252"(serine-type endopeptidase activity)	chain-seq-start:749|chain-seq-end:1663	chain-seq-start:19|chain-seq-end:1231	figure legend:Supp. fig 2A,  lane 3|comment:Factor I cleaves the a'  chain of the C3b complex.|comment:CFHR4A does not display cofactor activity, it enhances the cofactor activity of FH.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	rigid:JXnXHHpruX8l90QavbpphKaXO0I	false	-	-	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P01024-PRO_0000005911	uniprotkb:Q92496-1	intact:EBI-12735725	intact:EBI-22033617|ensembl:ENSP00000477162	psi-mi:p01024-pro_0000005911(display_long)|uniprotkb:C3(gene name)|psi-mi:C3(display_short)|uniprotkb:CPAMD1(gene name synonym)|uniprotkb:C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1(gene name synonym)	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:"MI:0435"(protease assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118441|imex:IM-27449-39	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:2274"(regulator)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-905851(chain-parent)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	go:"GO:0004252"(serine-type endopeptidase activity)	chain-seq-start:749|chain-seq-end:1663	-	figure legend:Supp. fig 2A,  lane 3|comment:Factor I cleaves the a'  chain of the C3b complex.|comment:CFHR4A does not display cofactor activity, it enhances the cofactor activity of FH.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/11/20	rogid:PGQNhqtLcK90Rf1IY85ARmU6Jbs9606	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rigid:JXnXHHpruX8l90QavbpphKaXO0I	false	-	his tag:c-c	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P00751-PRO_0000027547	uniprotkb:P08603-PRO_0000005894	intact:EBI-21988629	intact:EBI-22114230	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118270|imex:IM-27449-47	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:2274"(regulator)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-1223668(chain-parent)	intact:EBI-1223708(chain-parent)	-	chain-seq-start:260|chain-seq-end:764	chain-seq-start:19|chain-seq-end:1231	figure legend:Figure 5A/B, Supp fig 2B/C|comment:The CFHR4A-C3bBb convertase showed resistance against the decay accelerated by FH as compared with the C3bBb convertase.  Intact convertases were detected with anti-FB|comment:"The 'C3 convertase' has two components:- the C3b complex,  and \"Bb\" from Factor B. Uniprot states:-\"Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase\"."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/29	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	false	binding-associated region:?-?	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:P00751-PRO_0000027547	matrixdb:MULT_84_human	intact:EBI-21988629	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118270|imex:IM-27449-47	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-1223668(chain-parent)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	chain-seq-start:260|chain-seq-end:764	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:Figure 5A/B, Supp fig 2B/C|comment:The CFHR4A-C3bBb convertase showed resistance against the decay accelerated by FH as compared with the C3bBb convertase.  Intact convertases were detected with anti-FB|comment:"The 'C3 convertase' has two components:- the C3b complex,  and \"Bb\" from Factor B. Uniprot states:-\"Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase\"."|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/29	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	-	false	binding-associated region:?-?	binding-associated region:?-?	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P08603-PRO_0000005894	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-22114230	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:p08603-pro_0000005894(display_long)|uniprotkb:CFH(gene name)|psi-mi:CFH(display_short)|uniprotkb:HF(gene name synonym)|uniprotkb:HF1(gene name synonym)|uniprotkb:HF2(gene name synonym)|uniprotkb:H factor 1(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118276|imex:IM-27449-29	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:2274"(regulator)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	intact:EBI-1223708(chain-parent)	-	-	chain-seq-start:19|chain-seq-end:1231	figure legend:5A/B|comment:The CFHR4A-C3bBb convertase showed resistance against the decay accelerated by FH as compared with the C3bBb convertase.  Intact convertases were detected with anti-FB.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/29	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:XBVVm1S9GOtP5be24RqAkVBULzQ9606	-	false	binding-associated region:?-?	-	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	uniprotkb:P00751-PRO_0000027547	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-21988629	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:p00751-pro_0000027547(display_long)|uniprotkb:CFB(gene name)|psi-mi:CFB(display_short)|uniprotkb:BF(gene name synonym)|uniprotkb:BFD(gene name synonym)|uniprotkb:C3/C5 convertase(gene name synonym)|uniprotkb:Glycine-rich beta glycoprotein(gene name synonym)|uniprotkb:PBF2(gene name synonym)|uniprotkb:Properdin factor B(gene name synonym)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118276|imex:IM-27449-29	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	intact:EBI-1223668(chain-parent)	-	-	chain-seq-start:260|chain-seq-end:764	figure legend:5A/B|comment:The CFHR4A-C3bBb convertase showed resistance against the decay accelerated by FH as compared with the C3bBb convertase.  Intact convertases were detected with anti-FB.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/29	rogid:R26JtE6wIE5cqI97425F99Masjo9606	rogid:INYV2BPbjbUsThnuF2mPvKEn/iA9606	-	false	binding-associated region:?-?	binding-associated region:?-?	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)
uniprotkb:Q92496-1	matrixdb:MULT_84_human	intact:EBI-22033617|ensembl:ENSP00000477162	intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832	psi-mi:q92496-1(display_long)|uniprotkb:CFHR4(gene name)|psi-mi:CFHR4(display_short)|uniprotkb:CFHL4(gene name synonym)|uniprotkb:FHR4(gene name synonym)|uniprotkb:FHR-4A(isoform synonym)	psi-mi:c3b_human(display_short)|psi-mi:MULT_84_human(display_long)|intact:Complement C3b complex(complex recommended name)|intact:C3b dimer(complex systematic name)	psi-mi:"MI:0411"(enzyme linked immunosorbent assay)	Hebecker et al. (2012)	imex:IM-27449|pubmed:22518841	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-22118276|imex:IM-27449-29	-	psi-mi:"MI:1060"(spoke expansion)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:1302"(stable complex)	intact:EBI-2116399(isoform-parent)|ensembl:ENSG00000134365(gene)|ensembl:ENST00000608469(transcript)	pubmed:27782329(see-also)|pubmed:6225800(see-also)|evidence ontology:"ECO:0000353"|efo:"Orphanet:280133"(see-also)|efo:"Orphanet:2134"(see-also)|efo:"Orphanet:93575"(see-also)|efo:"EFO:0001365"(see-also)|pubmed:27013439(see-also)|pubmed:19196712(see-also)|pubmed:17051160(see-also)|go:"GO:0005615"(extracellular space)|go:"GO:0001848"(complement binding)|go:"GO:1903028"(positive regulation of opsonization)|go:"GO:0006956"(complement activation)|go:"GO:0050766"(positive regulation of phagocytosis)|go:"GO:0044533"(positive regulation of apoptotic process in another organism)|go:"GO:0050778"(positive regulation of immune response)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0008228"(opsonization)|pubmed:6559201(see-also)|pubmed:15454921(see-also)|pubmed:21205667(see-also)|pubmed:17051150(see-also)|complex portal:CPX-973(complex-primary)|pubmed:26489954(see-also)	-	-	curated-complex:"A protein complex of the alternative pathway of complement activation of the innate immune system. Complement C3 precurser is activated by cleavage into C3a and C3b by C3 convertases, either C4bC2a in the classical and lectin pathways or C3(H2O)Bb, C3bBb, C3bBbC3b, C3bBbP or C3bBbC3bP in the alternative pathway.     C3b acts as an opsonin and interacts with glycoproteins and carbohydrates on pathogenic or apoptotic target cell surfaces through its reactive thioester moiety. Opsonization of target cells leads to enhanced phagocytosis, lysis of target cells via membrane attack complex (CPX-6159) assembly, clearance of antibody-antigen complexes and up-regulation of the adaptive response.     Activation of C3b leads to an amplification cascade that generates more C3 convertase, deposits more C3b at the local site and switches C3 convertases to C5 convertases.     To protect host cells from inadvertent complement activation the activation of C3b is tightly regulated by either disrupting the C3 convertases or aiding in the proteolytic degradation of C3b. Bacteria and viruses possess C3b proteases to evade the complement response.     Lack of protection, due to familial mutations in the complement genes or the presence of autoantibodies against regulators has been linked to atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathies (C3G) in kidneys and age-related macular degeneration (AMD) in eyes. Conditions of chronic and acute inflammations, as in rheumatoid arthritis, strokes, and heart attacks, become aggravated by complement activation against the disturbed tissue."|complex-properties:"The alternative pathway is continuously activated at a low level. C3 precursor is first processed by the removal of 4 Arg residues and spontaneous hydrolysis due to the breakdown of the internal thioester bond forms C3(H2O) (disulfide bonded P01024-PRO_0000005909 & P01024-PRO_0000005908).     C3(H2O) subsequently binds factor B that is cleaved by Factor D (P00746) into 2 fragments: CFBa and CFBb. CFBb, a serine protease, then combines with complement factor C3(H2O) or C3b to generate the C3 or C5 convertases.     Proteolytic cleavage of C3 to C3b is accompanied by large conformational changes that result in the exposure of cryptic binding sites required for convertase assembly and regulation. This includes a significant movement of the thioester-bond-containing domain through which C3b attaches to target surfaces."|complex-assembly:Heterodimer|disease:"Complement component 3 (C3) deficiency [Orphanet:280133]: a rare, autosomal recessive defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis."|disease:"Atypical hemolytic-uremic syndrome with C3 anomaly [Orphanet:2134 & Orphanet:93575]: an autosomal dominant, atypical form of hemolytic-uremic syndrome (aHUS, a thrombotic microangiopathy) characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction."|disease:"Age-related macular degeneration [EFO:0001365]: age-related macular degeneration usually in older adults which results in a loss of vision in the center of the visual field (the macula lutea) because of damage to the retina. Manifests as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Occurs in dry and wet forms. Most common cause of irreversible vision loss in the developed world."	figure legend:5A/B|comment:The CFHR4A-C3bBb convertase showed resistance against the decay accelerated by FH as compared with the C3bBb convertase.  Intact convertases were detected with anti-FB.|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/09/20	2019/10/29	rogid:R26JtE6wIE5cqI97425F99Masjo9606	-	-	false	binding-associated region:?-?	binding-associated region:?-?|binding-associated region:?-?	-	-	psi-mi:"MI:0421"(identification by antibody)	psi-mi:"MI:0421"(identification by antibody)