#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:P09871	uniprotkb:P00736	intact:EBI-2810045|uniprotkb:Q9UCU9|uniprotkb:Q9UCV3|uniprotkb:Q9UCU7|uniprotkb:Q9UCV5|uniprotkb:Q9UCV4|uniprotkb:Q9UCU8|uniprotkb:Q9UCV2|uniprotkb:Q9UCV1|uniprotkb:Q9UCV0|uniprotkb:Q9UM14|uniprotkb:D3DUT4|ensembl:ENSP00000328173|ensembl:ENSP00000354057|ensembl:ENSP00000385035	intact:EBI-3926504|uniprotkb:Q8J012|uniprotkb:Q68D77|uniprotkb:A6NJQ8	psi-mi:c1s_human(display_long)|uniprotkb:C1S(gene name)|psi-mi:C1S(display_short)|uniprotkb:C1 esterase(gene name synonym)|uniprotkb:Complement component 1 subcomponent s(gene name synonym)	psi-mi:c1r_human(display_long)|uniprotkb:C1R(gene name)|psi-mi:C1R(display_short)|uniprotkb:Complement component 1 subcomponent r(gene name synonym)	psi-mi:"MI:0071"(molecular sieving)	Gröbner et al. (2018)	pubmed:31749804|imex:IM-27712	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-25425593|imex:IM-27712-1	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	refseq:NP_001725.1|refseq:NP_958850.1|ensembl:ENSG00000182326(gene)|ensembl:ENST00000328916(transcript)|ensembl:ENST00000360817(transcript)|ensembl:ENST00000406697(transcript)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0072562"(blood microparticle)|interpro:IPR000152(Aspartic acid and asparagine hydroxylation site)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR000859(CUB)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR018097|interpro:IPR033116|interpro:IPR035708|interpro:IPR035914|interpro:IPR035976|interpro:IPR043504|rcsb pdb:1ELV|rcsb pdb:1NZI|rcsb pdb:4J1Y|rcsb pdb:4LMF|rcsb pdb:4LOR|rcsb pdb:4LOS|rcsb pdb:4LOT|rcsb pdb:6F1C|rcsb pdb:6F1H|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|refseq:NP_001333779.1|refseq:XP_005253817.1	interpro:IPR035914|interpro:IPR035976|rcsb pdb:6F1C|rcsb pdb:6F1D|rcsb pdb:6F1H|refseq:NP_001724.3|interpro:IPR000859(CUB)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR018097|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR000436(Sushi/SCR/CCP)|rcsb pdb:1APQ|rcsb pdb:1GPZ|rcsb pdb:1MD7|rcsb pdb:1MD8|rcsb pdb:2QY0|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006955"(immune response)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0008236"(serine-type peptidase activity)|go:"GO:0031638"(zymogen activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR033116|rcsb pdb:6F39|interpro:IPR000742(EGF-like, type 3)|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|interpro:IPR035707|interpro:IPR043504	-	-	-	figure legend:Fig. 3A|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2020/02/13	2020/02/13	rogid:3zz5RwIqLyPO71zdCp5Vx7aVl+Y9606	rogid:7E+ASC6Hv3CoXdwfWEMwCtYALbw9606	rigid:SVO5OQAAwrsfd5kFxQT4EPmntbY	false	flag tag:c-c	strep tag:c-c|mutation:654-654|mutation with no effect:290-290	2	2	psi-mi:"MI:0396"(predetermined participant)	psi-mi:"MI:0396"(predetermined participant)
uniprotkb:P00736	uniprotkb:P00736	intact:EBI-3926504|uniprotkb:Q8J012|uniprotkb:Q68D77|uniprotkb:A6NJQ8	intact:EBI-3926504|uniprotkb:Q8J012|uniprotkb:Q68D77|uniprotkb:A6NJQ8	psi-mi:c1r_human(display_long)|uniprotkb:C1R(gene name)|psi-mi:C1R(display_short)|uniprotkb:Complement component 1 subcomponent r(gene name synonym)	psi-mi:c1r_human(display_long)|uniprotkb:C1R(gene name)|psi-mi:C1R(display_short)|uniprotkb:Complement component 1 subcomponent r(gene name synonym)	psi-mi:"MI:0071"(molecular sieving)	Gröbner et al. (2018)	pubmed:31749804|imex:IM-27712	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-25425694|imex:IM-27712-3	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	interpro:IPR035914|interpro:IPR035976|rcsb pdb:6F1C|rcsb pdb:6F1D|rcsb pdb:6F1H|refseq:NP_001724.3|interpro:IPR000859(CUB)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR018097|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR000436(Sushi/SCR/CCP)|rcsb pdb:1APQ|rcsb pdb:1GPZ|rcsb pdb:1MD7|rcsb pdb:1MD8|rcsb pdb:2QY0|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006955"(immune response)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0008236"(serine-type peptidase activity)|go:"GO:0031638"(zymogen activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR033116|rcsb pdb:6F39|interpro:IPR000742(EGF-like, type 3)|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|interpro:IPR035707|interpro:IPR043504	interpro:IPR035914|interpro:IPR035976|rcsb pdb:6F1C|rcsb pdb:6F1D|rcsb pdb:6F1H|refseq:NP_001724.3|interpro:IPR000859(CUB)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR018097|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR000436(Sushi/SCR/CCP)|rcsb pdb:1APQ|rcsb pdb:1GPZ|rcsb pdb:1MD7|rcsb pdb:1MD8|rcsb pdb:2QY0|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006955"(immune response)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0008236"(serine-type peptidase activity)|go:"GO:0031638"(zymogen activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR033116|rcsb pdb:6F39|interpro:IPR000742(EGF-like, type 3)|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|interpro:IPR035707|interpro:IPR043504	-	-	-	figure legend:Fig. 3A|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2020/02/13	2020/02/13	rogid:7E+ASC6Hv3CoXdwfWEMwCtYALbw9606	rogid:7E+ASC6Hv3CoXdwfWEMwCtYALbw9606	rigid:GE77+Rdb5/dCX72rWTJXxcYZQuc	false	strep tag:c-c|mutation:654-654|mutation with no effect:290-290	strep tag:c-c|mutation:654-654|mutation with no effect:290-290	2	0	psi-mi:"MI:0396"(predetermined participant)	psi-mi:"MI:0396"(predetermined participant)
matrixdb:MULT_82_human	uniprotkb:P09871	intact:EBI-6264901|reactome:R-HSA-173579|wwpdb:1PK6|wwpdb:2wnv|wwpdb:2jg8|wwpdb:2jg9|wwpdb:2wnu|reactome:R-HSA-173588	intact:EBI-2810045|uniprotkb:Q9UCU9|uniprotkb:Q9UCV3|uniprotkb:Q9UCU7|uniprotkb:Q9UCV5|uniprotkb:Q9UCV4|uniprotkb:Q9UCU8|uniprotkb:Q9UCV2|uniprotkb:Q9UCV1|uniprotkb:Q9UCV0|uniprotkb:Q9UM14|uniprotkb:D3DUT4|ensembl:ENSP00000328173|ensembl:ENSP00000354057|ensembl:ENSP00000385035	psi-mi:c1q_human(display_short)|psi-mi:MULT_82_human(display_long)|intact:Complement component C1q complex(complex recommended name)|intact:C1q(complex synonym)|intact:Complement 1q(complex synonym)|intact:C1QA-C1QB-C1QC complex(complex synonym)|intact:"6xC1QA:6xC1QB:6xC1QC"(complex systematic name)	psi-mi:c1s_human(display_long)|uniprotkb:C1S(gene name)|psi-mi:C1S(display_short)|uniprotkb:C1 esterase(gene name synonym)|uniprotkb:Complement component 1 subcomponent s(gene name synonym)	psi-mi:"MI:0107"(surface plasmon resonance)	Gröbner et al. (2018)	pubmed:31749804|imex:IM-27712	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-25425753|imex:IM-27712-5	-	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:1302"(stable complex)	psi-mi:"MI:0326"(protein)	go:"GO:0005509"(calcium ion binding)|go:"GO:0019865"(immunoglobulin binding)|efo:"MONDO:0013343"(see-also)|pubmed:28601358(see-also)|pubmed:22536204(see-also)|intact:EBI-6264878(exp-evidence)|evidence ontology:"ECO:0000353"|complex portal:CPX-1919(complex-primary)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0005576"(extracellular region)|pubmed:20548024(see-also)|pubmed:29449492(see-also)|pubmed:18250442(see-also)|go:"GO:0062167"(complement component C1q complex)|pubmed:31165008(see-also)|pubmed:27914690(see-also)|pubmed:26489954(see-also)	refseq:NP_001725.1|refseq:NP_958850.1|ensembl:ENSG00000182326(gene)|ensembl:ENST00000328916(transcript)|ensembl:ENST00000360817(transcript)|ensembl:ENST00000406697(transcript)|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0072562"(blood microparticle)|interpro:IPR000152(Aspartic acid and asparagine hydroxylation site)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR000859(CUB)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR018097|interpro:IPR033116|interpro:IPR035708|interpro:IPR035914|interpro:IPR035976|interpro:IPR043504|rcsb pdb:1ELV|rcsb pdb:1NZI|rcsb pdb:4J1Y|rcsb pdb:4LMF|rcsb pdb:4LOR|rcsb pdb:4LOS|rcsb pdb:4LOT|rcsb pdb:6F1C|rcsb pdb:6F1H|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|refseq:NP_001333779.1|refseq:XP_005253817.1	-	disease:"Complement component C1q deficiency (C1QD) [MONDO:0013343]: a disorder caused by impaired activation of the complement classical pathway. It generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis."|complex-properties:The complex comprises of six heterotrimeric collagen-like triple helices, formed from triplets of the A, B, and C chains, that associate in their N-terminal half to form a stalk, then diverge to form individual stems, each terminating in a C-terminal globular domain. Each of the six heterotrimeric globular heads or domains is made up of the globular domains from one A, one B, and one C chain, each of which in turn has its own ligand specificity. The six collagen-like stalks appear to be fibril-like and exist in the central region. The collagen-like region and the globular heads can independently interact with a multiplicity of biological structures including pathogen-associated and cell associated molecules, also each of the individual globular head domains is capable of independently interacting with specific ligands. Chains A and B are disulfide-linked whilst the C chain forms a disulfide bond with the C chain of the next strand.     Molecular weight 460 kDa.|curated-complex:"Recognition unit of the classical pathway of complement which associates with the Ca2+ - dependent C1r(2)-C1s(2) tetramer to form C1 (CPX-1920), the first component of the classical complement system. The complex's role in C1 is the recognition of immune complexes, or other molecules, which trigger the classical pathway of the complement system. Independent of complement activation C1q appears to have additional roles in homeostasis and cellular development, superoxide (O2-) production by neutrophils, blood coagulation and neurological synapse pruning."|complex-assembly:Heterooctadecamer	-	figure legend:Fig. 3B|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2020/02/13	2020/02/17	-	rogid:3zz5RwIqLyPO71zdCp5Vx7aVl+Y9606	-	false	-	flag tag:c-c	-	2	psi-mi:"MI:0396"(predetermined participant)	psi-mi:"MI:0396"(predetermined participant)
matrixdb:MULT_82_human	uniprotkb:P00736	intact:EBI-6264901|reactome:R-HSA-173579|wwpdb:1PK6|wwpdb:2wnv|wwpdb:2jg8|wwpdb:2jg9|wwpdb:2wnu|reactome:R-HSA-173588	intact:EBI-3926504|uniprotkb:Q8J012|uniprotkb:Q68D77|uniprotkb:A6NJQ8	psi-mi:c1q_human(display_short)|psi-mi:MULT_82_human(display_long)|intact:Complement component C1q complex(complex recommended name)|intact:C1q(complex synonym)|intact:Complement 1q(complex synonym)|intact:C1QA-C1QB-C1QC complex(complex synonym)|intact:"6xC1QA:6xC1QB:6xC1QC"(complex systematic name)	psi-mi:c1r_human(display_long)|uniprotkb:C1R(gene name)|psi-mi:C1R(display_short)|uniprotkb:Complement component 1 subcomponent r(gene name synonym)	psi-mi:"MI:0107"(surface plasmon resonance)	Gröbner et al. (2018)	pubmed:31749804|imex:IM-27712	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-25425753|imex:IM-27712-5	-	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:1302"(stable complex)	psi-mi:"MI:0326"(protein)	go:"GO:0005509"(calcium ion binding)|go:"GO:0019865"(immunoglobulin binding)|efo:"MONDO:0013343"(see-also)|pubmed:28601358(see-also)|pubmed:22536204(see-also)|intact:EBI-6264878(exp-evidence)|evidence ontology:"ECO:0000353"|complex portal:CPX-1919(complex-primary)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0005576"(extracellular region)|pubmed:20548024(see-also)|pubmed:29449492(see-also)|pubmed:18250442(see-also)|go:"GO:0062167"(complement component C1q complex)|pubmed:31165008(see-also)|pubmed:27914690(see-also)|pubmed:26489954(see-also)	interpro:IPR035914|interpro:IPR035976|rcsb pdb:6F1C|rcsb pdb:6F1D|rcsb pdb:6F1H|refseq:NP_001724.3|interpro:IPR000859(CUB)|interpro:IPR001881(EGF-like calcium-binding)|interpro:IPR018097|interpro:IPR009003(Peptidase, trypsin-like serine and cysteine)|interpro:IPR001254(Peptidase S1 and S6, chymotrypsin/Hap)|interpro:IPR001314(Peptidase S1A, chymotrypsin)|interpro:IPR000436(Sushi/SCR/CCP)|rcsb pdb:1APQ|rcsb pdb:1GPZ|rcsb pdb:1MD7|rcsb pdb:1MD8|rcsb pdb:2QY0|go:"GO:0004252"(serine-type endopeptidase activity)|go:"GO:0005509"(calcium ion binding)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006955"(immune response)|go:"GO:0006958"(complement activation, classical pathway)|go:"GO:0008236"(serine-type peptidase activity)|go:"GO:0031638"(zymogen activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0045087"(innate immune response)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|interpro:IPR033116|rcsb pdb:6F39|interpro:IPR000742(EGF-like, type 3)|reactome:R-HSA-166663|reactome:R-HSA-173623|reactome:R-HSA-977606|interpro:IPR035707|interpro:IPR043504	-	disease:"Complement component C1q deficiency (C1QD) [MONDO:0013343]: a disorder caused by impaired activation of the complement classical pathway. It generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis."|complex-properties:The complex comprises of six heterotrimeric collagen-like triple helices, formed from triplets of the A, B, and C chains, that associate in their N-terminal half to form a stalk, then diverge to form individual stems, each terminating in a C-terminal globular domain. Each of the six heterotrimeric globular heads or domains is made up of the globular domains from one A, one B, and one C chain, each of which in turn has its own ligand specificity. The six collagen-like stalks appear to be fibril-like and exist in the central region. The collagen-like region and the globular heads can independently interact with a multiplicity of biological structures including pathogen-associated and cell associated molecules, also each of the individual globular head domains is capable of independently interacting with specific ligands. Chains A and B are disulfide-linked whilst the C chain forms a disulfide bond with the C chain of the next strand.     Molecular weight 460 kDa.|curated-complex:"Recognition unit of the classical pathway of complement which associates with the Ca2+ - dependent C1r(2)-C1s(2) tetramer to form C1 (CPX-1920), the first component of the classical complement system. The complex's role in C1 is the recognition of immune complexes, or other molecules, which trigger the classical pathway of the complement system. Independent of complement activation C1q appears to have additional roles in homeostasis and cellular development, superoxide (O2-) production by neutrophils, blood coagulation and neurological synapse pruning."|complex-assembly:Heterooctadecamer	-	figure legend:Fig. 3B|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2020/02/13	2020/02/17	-	rogid:7E+ASC6Hv3CoXdwfWEMwCtYALbw9606	-	false	-	mutation:654-654|mutation disrupting interaction strength:290-290|strep tag:c-c	-	2	psi-mi:"MI:0396"(predetermined participant)	psi-mi:"MI:0396"(predetermined participant)