#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:P36980 uniprotkb:Q03591 intact:EBI-21976709|uniprotkb:Q14310|uniprotkb:Q5T9T1|ensembl:ENSP00000356385 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr2_human(display_long)|uniprotkb:CFHR2(gene name)|psi-mi:CFHR2(display_short)|uniprotkb:CFHL2(gene name synonym)|uniprotkb:DDESK59(gene name synonym)|uniprotkb:FHR2(gene name synonym)|uniprotkb:H factor-like 3(gene name synonym)|uniprotkb:H factor-like protein 2(gene name synonym)|uniprotkb:HFL3(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0004"(affinity chromatography technology) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016705|imex:IM-27343-1 - - 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_001299601.1|refseq:NP_005657.1|ensembl:ENST00000367415(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|rcsb pdb:3ZD1|rcsb pdb:5EA0|reactome:R-HSA-977606|ensembl:ENSG00000080910(gene) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 3, 5A, Supplementary figure 5.|comment:Identical results were obtained with FH-depleted plasma from individuals with each of the 3 CFHR1*A/B genotypes.|comment:"Results obtained from the FH-depleted plasma of individual GN29M (with an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5, and also heterozygous for a deletion of the CFHR3 and CFHR1 genes) showed an abnormal profile, with the mutant FHR1 coeluting with FHR2 through most of the NaCl gradient."|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/09/12 rogid:dYw4bCXCH0UiK22QrAf1ApfZE/s9606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:agOIhepJy/g3TLvwXA696SqVVRI false - mutation with no effect:157-157,159-159,175-175 - - psi-mi:"MI:0816"(molecular weight estimation by staining) psi-mi:"MI:0816"(molecular weight estimation by staining) uniprotkb:Q9BXR6 uniprotkb:Q03591 intact:EBI-11579371|uniprotkb:Q2NKK2|ensembl:ENSP00000256785 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr5_human(display_long)|uniprotkb:CFHR5(gene name)|psi-mi:CFHR5(display_short)|uniprotkb:CFHL5(gene name synonym)|uniprotkb:FHR5(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0004"(affinity chromatography technology) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016727|imex:IM-27343-3 - - 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) refseq:NP_110414.1|ensembl:ENSG00000134389(gene)|ensembl:ENST00000256785(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|reactome:R-HSA-977606 ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 3, Supplementary figure 5.|comment:Identical results were obtained with FH-depleted plasma from individuals with each of the 3 CFHR1*A/B genotypes.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/09/12 rogid:+67VN78vOOCn/pY6CMAZS21XGv49606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:noo8nYWC6bXZFvsjEkz884X/N+Y false - mutation with no effect:157-157,159-159,175-175 - - psi-mi:"MI:0816"(molecular weight estimation by staining) psi-mi:"MI:0816"(molecular weight estimation by staining) uniprotkb:Q9BXR6 uniprotkb:Q03591 intact:EBI-11579371|uniprotkb:Q2NKK2|ensembl:ENSP00000256785 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr5_human(display_long)|uniprotkb:CFHR5(gene name)|psi-mi:CFHR5(display_short)|uniprotkb:CFHL5(gene name synonym)|uniprotkb:FHR5(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0276"(blue native page) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016697|imex:IM-27343-21 - - 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_110414.1|ensembl:ENSG00000134389(gene)|ensembl:ENST00000256785(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|reactome:R-HSA-977606 ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 4 A/B, Lanes 12 + 13.|comment:Lanes 12 and 13 corresponded to different FHR1-FHR5 hetero-oligomers.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:+67VN78vOOCn/pY6CMAZS21XGv49606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:noo8nYWC6bXZFvsjEkz884X/N+Y false - - - - psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:P36980 uniprotkb:Q03591 intact:EBI-21976709|uniprotkb:Q14310|uniprotkb:Q5T9T1|ensembl:ENSP00000356385 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr2_human(display_long)|uniprotkb:CFHR2(gene name)|psi-mi:CFHR2(display_short)|uniprotkb:CFHL2(gene name synonym)|uniprotkb:DDESK59(gene name synonym)|uniprotkb:FHR2(gene name synonym)|uniprotkb:H factor-like 3(gene name synonym)|uniprotkb:H factor-like protein 2(gene name synonym)|uniprotkb:HFL3(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0276"(blue native page) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016736|imex:IM-27343-23 - - 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_001299601.1|refseq:NP_005657.1|ensembl:ENST00000367415(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|rcsb pdb:3ZD1|rcsb pdb:5EA0|reactome:R-HSA-977606|ensembl:ENSG00000080910(gene) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 4A/B, Lane 7|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:dYw4bCXCH0UiK22QrAf1ApfZE/s9606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:agOIhepJy/g3TLvwXA696SqVVRI false - - 1 1 psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:Q03591 uniprotkb:Q03591 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0276"(blue native page) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016747|imex:IM-27343-25 - - 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) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 4A/B, Lane 1+2|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:bEve3SUg9q4GC0vX6Ea0hXCPDus false - - 2 0 psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:Q9BXR6 uniprotkb:Q9BXR6 intact:EBI-11579371|uniprotkb:Q2NKK2|ensembl:ENSP00000256785 intact:EBI-11579371|uniprotkb:Q2NKK2|ensembl:ENSP00000256785 psi-mi:fhr5_human(display_long)|uniprotkb:CFHR5(gene name)|psi-mi:CFHR5(display_short)|uniprotkb:CFHL5(gene name synonym)|uniprotkb:FHR5(gene name synonym) psi-mi:fhr5_human(display_long)|uniprotkb:CFHR5(gene name)|psi-mi:CFHR5(display_short)|uniprotkb:CFHL5(gene name synonym)|uniprotkb:FHR5(gene name synonym) psi-mi:"MI:0276"(blue native page) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016751|imex:IM-27343-27 - - 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_110414.1|ensembl:ENSG00000134389(gene)|ensembl:ENST00000256785(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|reactome:R-HSA-977606 refseq:NP_110414.1|ensembl:ENSG00000134389(gene)|ensembl:ENST00000256785(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|reactome:R-HSA-977606 - - - figure legend:Figure 4 A/B, Lane 4|comment:FHR2 and FHR5 only formed homo-oligomers in the absence of FHR1.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:+67VN78vOOCn/pY6CMAZS21XGv49606 rogid:+67VN78vOOCn/pY6CMAZS21XGv49606 rigid:pMfNJ3+reWjbVAS/qegKRehs/h0 false - - 2 0 psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:P36980 uniprotkb:P36980 intact:EBI-21976709|uniprotkb:Q14310|uniprotkb:Q5T9T1|ensembl:ENSP00000356385 intact:EBI-21976709|uniprotkb:Q14310|uniprotkb:Q5T9T1|ensembl:ENSP00000356385 psi-mi:fhr2_human(display_long)|uniprotkb:CFHR2(gene name)|psi-mi:CFHR2(display_short)|uniprotkb:CFHL2(gene name synonym)|uniprotkb:DDESK59(gene name synonym)|uniprotkb:FHR2(gene name synonym)|uniprotkb:H factor-like 3(gene name synonym)|uniprotkb:H factor-like protein 2(gene name synonym)|uniprotkb:HFL3(gene name synonym) psi-mi:fhr2_human(display_long)|uniprotkb:CFHR2(gene name)|psi-mi:CFHR2(display_short)|uniprotkb:CFHL2(gene name synonym)|uniprotkb:DDESK59(gene name synonym)|uniprotkb:FHR2(gene name synonym)|uniprotkb:H factor-like 3(gene name synonym)|uniprotkb:H factor-like protein 2(gene name synonym)|uniprotkb:HFL3(gene name synonym) psi-mi:"MI:0276"(blue native page) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016756|imex:IM-27343-29 - - 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_001299601.1|refseq:NP_005657.1|ensembl:ENST00000367415(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|rcsb pdb:3ZD1|rcsb pdb:5EA0|reactome:R-HSA-977606|ensembl:ENSG00000080910(gene) refseq:NP_001299601.1|refseq:NP_005657.1|ensembl:ENST00000367415(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|rcsb pdb:3ZD1|rcsb pdb:5EA0|reactome:R-HSA-977606|ensembl:ENSG00000080910(gene) - - - figure legend:Figure 4 A/B, Lane 5|comment:FHR2 and FHR5 only formed homo-oligomers in the absence of FHR1.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:dYw4bCXCH0UiK22QrAf1ApfZE/s9606 rogid:dYw4bCXCH0UiK22QrAf1ApfZE/s9606 rigid:NwB2keU6AKkDLQXN7xn7G/1qTZY false - - 2 0 psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:Q03591 uniprotkb:P36980 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-21976709|uniprotkb:Q14310|uniprotkb:Q5T9T1|ensembl:ENSP00000356385 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:fhr2_human(display_long)|uniprotkb:CFHR2(gene name)|psi-mi:CFHR2(display_short)|uniprotkb:CFHL2(gene name synonym)|uniprotkb:DDESK59(gene name synonym)|uniprotkb:FHR2(gene name synonym)|uniprotkb:H factor-like 3(gene name synonym)|uniprotkb:H factor-like protein 2(gene name synonym)|uniprotkb:HFL3(gene name synonym) psi-mi:"MI:0071"(molecular sieving) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016782|imex:IM-27343-5 - - 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) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 refseq:NP_001299601.1|refseq:NP_005657.1|ensembl:ENST00000367415(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|rcsb pdb:3ZD1|rcsb pdb:5EA0|reactome:R-HSA-977606|ensembl:ENSG00000080910(gene) - - - figure legend:Figure 5B, Supp. fig 8.|comment:"Results obtained from normal plasma and the FH-depleted plasma of individual GN29M (with an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5, and also heterozygous for a deletion of the CFHR3 and CFHR1 genes);peaks containing the mutant FHR1 protein, had a broader range and a higher molecular weight than that from normal plasma."|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/09/12 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rogid:dYw4bCXCH0UiK22QrAf1ApfZE/s9606 rigid:agOIhepJy/g3TLvwXA696SqVVRI false - - - - psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:Q03591 uniprotkb:Q9BXR6 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-11579371|uniprotkb:Q2NKK2|ensembl:ENSP00000256785 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:fhr5_human(display_long)|uniprotkb:CFHR5(gene name)|psi-mi:CFHR5(display_short)|uniprotkb:CFHL5(gene name synonym)|uniprotkb:FHR5(gene name synonym) psi-mi:"MI:0071"(molecular sieving) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016925|imex:IM-27343-15 - - 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) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 refseq:NP_110414.1|ensembl:ENSG00000134389(gene)|ensembl:ENST00000256785(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0051838"(cytolysis by host of symbiont cells)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|reactome:R-HSA-977606 - - - figure legend:Supp. fig 8.|comment:Results obtained from normal plasma.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/09/12 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rogid:+67VN78vOOCn/pY6CMAZS21XGv49606 rigid:noo8nYWC6bXZFvsjEkz884X/N+Y false - - - - psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) uniprotkb:Q03591 uniprotkb:Q03591 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0040"(electron microscopy) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016800|imex:IM-27343-7 - - 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) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Figure 6|comment:"A preparation enriched for the large-size mutant FHR1 complexes;the mutant FHR1 has an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5, and may also have a deletion of the CFHR3 and CFHR1 genes (not stated)."|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:9606(human-plasma)|taxid:9606(homo sapiens blood plasma) - 2019/08/20 2019/11/04 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rigid:6cYhsY8rwLQSGiswxOjqNmzUvIM false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) uniprotkb:Q03591 uniprotkb:P01024-PRO_0000005913 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-21988425 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:p01024-pro_0000005913(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:0107"(surface plasmon resonance) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016836|imex:IM-27343-9 - - 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:0326"(protein) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 intact:EBI-905851(chain-parent) - - chain-seq-start:955|chain-seq-end:1303 figure legend:Figure 7|comment:Mutant FHR1 bound more efficiently to C3dg than the native FHR1 protein.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/09/12 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 rogid:SVadmf2wemhTxgqZ2zqxo8bQHhU9606 rigid:bjavON8bdcP89Y0aLsAf90ws6t4 false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) uniprotkb:Q03591 matrixdb:MULT_84_human intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832 psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(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:0107"(surface plasmon resonance) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016857|imex:IM-27343-11 - - 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) ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 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:Figure 7|comment:Mutant FHR1 bound more efficiently to C3b and iC3b than the native FHR1 protein.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/10/07 rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 - - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) uniprotkb:P08603 matrixdb:MULT_84_human intact:EBI-1223708|uniprotkb:A5PL14|uniprotkb:Q2TAZ5|uniprotkb:P78435|uniprotkb:Q14570|uniprotkb:Q38G77|uniprotkb:Q5TFM3|uniprotkb:Q8N708|uniprotkb:Q9NU86|ensembl:ENSP00000356399 intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832 psi-mi:cfah_human(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:0107"(surface plasmon resonance) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22016906|imex:IM-27343-13 - - 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) refseq:NP_000177.2|dip:DIP-38303N|ensembl:ENSG00000000971(gene)|ensembl:ENST00000367429(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0008201"(heparin binding)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0043395"(heparan sulfate proteoglycan binding)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:1903659"(regulation of complement-dependent cytotoxicity)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:P08603|rcsb pdb:1FHC|rcsb pdb:1HAQ|rcsb pdb:1HCC|rcsb pdb:1HFH|rcsb pdb:1HFI|rcsb pdb:1KOV|rcsb pdb:2QFH|rcsb pdb:2RLP|rcsb pdb:2RLQ|rcsb pdb:2UWN|rcsb pdb:2V8E|rcsb pdb:2W80|rcsb pdb:2BZM|rcsb pdb:2G7I|rcsb pdb:2W81|rcsb pdb:2WII|rcsb pdb:2XQW|rcsb pdb:3GAU|rcsb pdb:3GAV|rcsb pdb:3GAW|rcsb pdb:3KXV|rcsb pdb:3KZJ|rcsb pdb:3OXU|rcsb pdb:3R62|rcsb pdb:3RJ3|rcsb pdb:3SW0|rcsb pdb:4AYD|rcsb pdb:4AYE|rcsb pdb:4AYI|rcsb pdb:4AYM|rcsb pdb:4B2R|rcsb pdb:4B2S|rcsb pdb:2IC4|rcsb pdb:2JGW|rcsb pdb:2JGX|rcsb pdb:4J38|rcsb pdb:4K12|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:2KMS|rcsb pdb:2QFG|rcsb pdb:5WTB|rcsb pdb:6ATG|reactome:R-HSA-977606 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:Figure 7B|comment:Conversion to iC3b almost eliminated binding of FH.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/10/07 rogid:1mjo+AJLIQnEsMFqsGsmUFbfUxk9606 - - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) uniprotkb:P00751 matrixdb:MULT_84_human 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 intact:EBI-12735745|wwpdb:5fo7|wwpdb:3g6j|wwpdb:2i07|reactome:R-HSA-166832 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: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:0107"(surface plasmon resonance) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 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-22017030|imex:IM-27343-17 - - 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) 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 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:Supplementary Figure 9|comment:AP proconvertase was formed.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/10/07 rogid:A6O1R+3K/DVzxKoEh2sLjEmDY/s9606 - - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) chebi:"CHEBI:28304" uniprotkb:Q03591 intact:EBI-1379423 intact:EBI-3935840|uniprotkb:Q9UJ17|uniprotkb:Q3B774|uniprotkb:A8K465|ensembl:ENSP00000314299 psi-mi:heparin(display_short)|psi-mi:"CHEBI:28304"(display_long) psi-mi:fhr1_human(display_long)|uniprotkb:CFHR1(gene name)|psi-mi:CFHR1(display_short)|uniprotkb:CFHL(gene name synonym)|uniprotkb:CFHL1(gene name synonym)|uniprotkb:CFHL1P(gene name synonym)|uniprotkb:CFHR1P(gene name synonym)|uniprotkb:FHR1(gene name synonym)|uniprotkb:HFL1(gene name synonym)|uniprotkb:HFL2(gene name synonym)|uniprotkb:H factor-like protein 1(gene name synonym)|uniprotkb:H36(gene name synonym) psi-mi:"MI:0004"(affinity chromatography technology) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 taxid:-2(chemical synthesis)|taxid:-2("Chemical synthesis (Chemical synthesis)") taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0914"(association) psi-mi:"MI:0469"(IntAct) intact:EBI-22016912|imex:IM-27343-31 - 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:0328"(small molecule) psi-mi:"MI:0326"(protein) - ensembl:ENSG00000244414(gene)|ensembl:ENST00000320493(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0032091"(negative regulation of protein binding)|go:"GO:0032991"(protein-containing complex)|go:"GO:0042802"(identical protein binding)|go:"GO:0051838"(cytolysis by host of symbiont cells)|go:"GO:0072562"(blood microparticle)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:Q03591|rcsb pdb:3ZD2|rcsb pdb:4MUC|reactome:R-HSA-977606|refseq:NP_002104.2 - - - figure legend:Supplementary Figure 2|comment:Plasma from patient GN29 with mutant FHR1 which has an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/09/12 - rogid:dXRmpm1kRulEmJS9dtU4ICqi24c9606 - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0818"(molecular weight estimation by coomasie staining)|psi-mi:"MI:0427"(Identification by mass spectrometry) chebi:"CHEBI:28304" uniprotkb:P08603 intact:EBI-1379423 intact:EBI-1223708|uniprotkb:A5PL14|uniprotkb:Q2TAZ5|uniprotkb:P78435|uniprotkb:Q14570|uniprotkb:Q38G77|uniprotkb:Q5TFM3|uniprotkb:Q8N708|uniprotkb:Q9NU86|ensembl:ENSP00000356399 psi-mi:heparin(display_short)|psi-mi:"CHEBI:28304"(display_long) psi-mi:cfah_human(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:0004"(affinity chromatography technology) Tortajada et al. (2013) imex:IM-27343|pubmed:23728178 taxid:-2(chemical synthesis)|taxid:-2("Chemical synthesis (Chemical synthesis)") taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0914"(association) psi-mi:"MI:0469"(IntAct) intact:EBI-22016912|imex:IM-27343-31 - 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:0328"(small molecule) psi-mi:"MI:0326"(protein) - refseq:NP_000177.2|dip:DIP-38303N|ensembl:ENSG00000000971(gene)|ensembl:ENST00000367429(transcript)|go:"GO:0005576"(extracellular region)|go:"GO:0005615"(extracellular space)|go:"GO:0006956"(complement activation)|go:"GO:0006957"(complement activation, alternative pathway)|go:"GO:0008201"(heparin binding)|go:"GO:0030449"(regulation of complement activation)|go:"GO:0042802"(identical protein binding)|go:"GO:0043395"(heparan sulfate proteoglycan binding)|go:"GO:0070062"(extracellular exosome)|go:"GO:0072562"(blood microparticle)|go:"GO:1903659"(regulation of complement-dependent cytotoxicity)|interpro:IPR000436(Sushi/SCR/CCP)|interpro:IPR035976|mint:P08603|rcsb pdb:1FHC|rcsb pdb:1HAQ|rcsb pdb:1HCC|rcsb pdb:1HFH|rcsb pdb:1HFI|rcsb pdb:1KOV|rcsb pdb:2QFH|rcsb pdb:2RLP|rcsb pdb:2RLQ|rcsb pdb:2UWN|rcsb pdb:2V8E|rcsb pdb:2W80|rcsb pdb:2BZM|rcsb pdb:2G7I|rcsb pdb:2W81|rcsb pdb:2WII|rcsb pdb:2XQW|rcsb pdb:3GAU|rcsb pdb:3GAV|rcsb pdb:3GAW|rcsb pdb:3KXV|rcsb pdb:3KZJ|rcsb pdb:3OXU|rcsb pdb:3R62|rcsb pdb:3RJ3|rcsb pdb:3SW0|rcsb pdb:4AYD|rcsb pdb:4AYE|rcsb pdb:4AYI|rcsb pdb:4AYM|rcsb pdb:4B2R|rcsb pdb:4B2S|rcsb pdb:2IC4|rcsb pdb:2JGW|rcsb pdb:2JGX|rcsb pdb:4J38|rcsb pdb:4K12|rcsb pdb:4ONT|rcsb pdb:4ZH1|rcsb pdb:5NBQ|rcsb pdb:5O32|rcsb pdb:5O35|rcsb pdb:2KMS|rcsb pdb:2QFG|rcsb pdb:5WTB|rcsb pdb:6ATG|reactome:R-HSA-977606 - - - figure legend:Supplementary Figure 2|comment:Plasma from patient GN29 with mutant FHR1 which has an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2019/08/20 2019/09/12 - rogid:1mjo+AJLIQnEsMFqsGsmUFbfUxk9606 - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0818"(molecular weight estimation by coomasie staining)|psi-mi:"MI:0427"(Identification by mass spectrometry)