IntAct European Bioinformatics Institute IntAct intact http://www.ebi.ac.uk/ http://www.ebi.ac.uk/intact/query/${ac} EBI-[0-9]+|IA:[0-9]+ INTerAction database (IntAct) provides an open source database and toolkit for the storage, presentation and analysis of molecular interactions. http://www.ebi.ac.uk/intact European Bioinformatics Institute; Wellcome Trust Genome Campus; Hinxton, Cambridge; CB10 1SD; United Kingdom http://www.ebi.ac.uk/intact/ C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation in vitro In vitro spr surface plasmon resonance Optical biosensor BIAcore(r) BIAcore(r) Optical biosensor spr predetermined predetermined participant predetermined Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. C3b is a complex composed of the modified C3-alpha chain (a') (M.W. 105,000) and the intact C3-beta chain (M.W. 75,000). Factor I cleaves the a' chain of the C3b complex. Native and mutant FHR1 were used in these assays;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. However it is not stated if the mutant FHR1 is from GM29M individual who is heterozgous for the deletion or, GN29, which has no deletion. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation human-plasma homo sapiens blood plasma plasma blood plasma bn-page blue native page bn-page western blot western blot Immuno blot Immuno blot Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation in vitro In vitro affinity chrom affinity chromatography technology Affinity purification Affinity purification affinity chrom experimental particp experimental participant identification experimental particp Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation Mock publication and experiment for abstract interactions that are not interaction evidences. Mock publication and experiment for abstract interactions that are not interaction evidences. unspecified method C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation human-plasma homo sapiens blood plasma plasma blood plasma electron microscopy electron microscopy Electron crystallography Electron cryomicroscopy Electron crystallography Electron cryomicroscopy predetermined predetermined participant predetermined Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation human-plasma homo sapiens blood plasma plasma blood plasma molecular sieving molecular sieving Gel Filtration Size Exclusion Chromatography Size Exclusion Chromatography Gel Filtration Sizing column western blot western blot Immuno blot Immuno blot Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. The Journal of clinical investigation 2013 imex curation Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es 04-05-20: Contacted by IntAct-Help. Only protein-protein interactions imex curation human-plasma homo sapiens blood plasma plasma blood plasma affinity chrom affinity chromatography technology Affinity purification Affinity purification affinity chrom weight by staining molecular weight estimation by staining weight by staining Tortajada A., Yébenes H., Abarrategui-Garrido C., Anter J., García-Fernández JM., Martínez-Barricarte R., Alba-Domínguez M., Malik TH., Bedoya R., Cabrera Pérez R., López Trascasa M., Pickering MC., Harris CL., Sánchez-Corral P., Llorca O., Rodríguez de Córdoba S. Details regarding 3 CFHR1*A/B genotypes were found in PMID:19745068- previous work of co-author C. Abarrategui-Garrido. i have removed the two ineractions/last experiment as requested! *********Factor I species:-this paper is Human throughout so I cannot think they would use mouse/rat proteins? If you do not like this I will remove Experiment 7. Heparin Affinity chromatography of plasma with a sodium chloride gradient; detection of proteins used a Mab that which recognizes an epitope common to FHR1, FHR2, and FHR5 (Figures 3 and 5) or an antibody that recognises FH (figure 1C and Supp. fig 2). No species origin is stated in this publication for Factor I, and the authors did not reply to a request for information;however it has been assumed to be of Human origin. SRdeCordoba@cib.csic.es Accepted 2020-FEB-12 AT 11:21 GMT AT 11:21 GMT by MARTA The Journal of clinical investigation 2013 Only protein-protein interactions imex curation imex curation p01024-pro_0000005908 Complement C3 beta chain C3 CPAMD1 C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1 protein protein human Homo sapiens Human SPMYSIITPNILRLESEETMVLEAHDAQGDVPVTVTVHDFPGKKLVLSSEKTVLTPATNHMGNVTFTIPANREFKSEKGRNKFVTVQATFGTQVVEKVVLVSLQSGYLFIQTDKTIYTPGSTVLYRIFTVNHKLLPVGRTVMVNIENPEGIPVKQDSLSSQNQLGVLPLSWDIPELVNMGQWKIRAYYENSPQQVFSTEFEVKEYVLPSFEVIVEPTEKFYYIYNEKGLEVTITARFLYGKKVEGTAFVIFGIQDGEQRISLPESLKRIPIEDGSGEVVLSRKVLLDGVQNPRAEDLVGKSLYVSATVILHSGSDMVQAERSGIPIVTSPYQIHFTKTPKYFKPGMPFDLMVFVTNPDGSPAYRVPVAVQGEDTVQSLTQGDGVAKLSINTHPSQKPLSITVRTKKQELSEAEQATRTMQALPYSTVGNSNNYLHLSVLRTELRPGETLNVNFLLRMDRAHEAKIRYYTYLIMNKGRLLKAGRQVREPGQDLVVLPLSITTDFIPSFRLVAYYTLIGASGQREVVADSVWVDVKDSCVGSLVVKSGQSEDRQPVPGQQMTLKIEGDHGARVVLVAVDKGVFVLNKKNKLTQSKIWDVVEKADIGCTPGSGKDYAGVFSDAGLTFTSSSGQQTAQRAELQCPQPAA 23 667 C4754001F1A97259 fhr2_human Complement factor H-related protein 2 CFHR2 CFHL2 DDESK59 FHR2 H factor-like 3 H factor-like protein 2 HFL3 protein protein human Homo sapiens Human MWLLVSVILISRISSVGGEAMFCDFPKINHGILYDEEKYKPFSQVPTGEVFYYSCEYNFVSPSKSFWTRITCAEEGWSPTPKCLRLCFFPFVENGHSESSGQTHLEGDTVQIICNTGYRLQNNENNISCVERGWSTPPKCRSTISAEKCGPPPPIDNGDITSFLLSVYAPGSSVEYQCQNLYQLEGNNQITCRNGQWSEPPKCLDPCVISQEIMEKYNIKLKWTNQQKLYSRTGDIVEFVCKSGYHPTKSHSFRAMCQNGKLVYPSCEEK E1B2E9F139B217A8 cfah_human Complement factor H CFH HF HF1 HF2 H factor 1 protein protein human Homo sapiens Human MRLLAKIICLMLWAICVAEDCNELPPRRNTEILTGSWSDQTYPEGTQAIYKCRPGYRSLGNVIMVCRKGEWVALNPLRKCQKRPCGHPGDTPFGTFTLTGGNVFEYGVKAVYTCNEGYQLLGEINYRECDTDGWTNDIPICEVVKCLPVTAPENGKIVSSAMEPDREYHFGQAVRFVCNSGYKIEGDEEMHCSDDGFWSKEKPKCVEISCKSPDVINGSPISQKIIYKENERFQYKCNMGYEYSERGDAVCTESGWRPLPSCEEKSCDNPYIPNGDYSPLRIKHRTGDEITYQCRNGFYPATRGNTAKCTSTGWIPAPRCTLKPCDYPDIKHGGLYHENMRRPYFPVAVGKYYSYYCDEHFETPSGSYWDHIHCTQDGWSPAVPCLRKCYFPYLENGYNQNYGRKFVQGKSIDVACHPGYALPKAQTTVTCMENGWSPTPRCIRVKTCSKSSIDIENGFISESQYTYALKEKAKYQCKLGYVTADGETSGSITCGKDGWSAQPTCIKSCDIPVFMNARTKNDFTWFKLNDTLDYECHDGYESNTGSTTGSIVCGYNGWSDLPICYERECELPKIDVHLVPDRKKDQYKVGEVLKFSCKPGFTIVGPNSVQCYHFGLSPDLPICKEQVQSCGPPPELLNGNVKEKTKEEYGHSEVVEYYCNPRFLMKGPNKIQCVDGEWTTLPVCIVEESTCGDIPELEHGWAQLSSPPYYYGDSVEFNCSESFTMIGHRSITCIHGVWTQLPQCVAIDKLKKCKSSNLIILEEHLKNKKEFDHNSNIRYRCRGKEGWIHTVCINGRWDPEVNCSMAQIQLCPPPPQIPNSHNMTTTLNYRDGEKVSVLCQENYLIQEGEEITCKDGRWQSIPLCVEKIPCSQPPQIEHGTINSSRSSQESYAHGTKLSYTCEGGFRISEENETTCYMGKWSSPPQCEGLPCKSPPEISHGVVAHMSDSYQYGEEVTYKCFEGFGIDGPAIAKCLGEKWSHPPSCIKTDCLSLPSFENAIPMGEKKDVYKAGEQVTYTCATYYKMDGASNVTCINSRWTGRPTCRDTSCVNPPTVQNAYIVSRQMSKYPSGERVRYQCRSPYEMFGDEEVMCLNGNWTEPPQCKDSTGKCGPPPPIDNGDITSFPLSVYAPASSVEYQCQNLYQLEGNKRITCRNGQWSEPPKCLHPCVISREIMENYNIALRWTAKQKLYSRTGESVEFVCKRGYRLSSRSHTLRTTCWDGKLEYPTCAKR DIP protein P08603 original sequence version: 161 DIP protein P08603-1 original sequence version: 149 3C26D62A2BF9BFEE p01024-pro_0000005913 Complement C3dg fragment C3 CPAMD1 C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1 protein protein human Homo sapiens Human EGVQKEDIPPADLSDQVPDTESETRILLQGTPVAQMTEDAVDAERLKHLIVTPSGCGEQNMIGMTPTVIAVHYLDETEQWEKFGLEKRQGALELIKKGYTQQLAFRQPSSAFAAFVKRAPSTWLTAYVVKVFSLAVNLIAIDSQVLCGAVKWLILEKQKPDGVFQEDAPVIHQEMIGGLRNNNEKDMALTAFVLISLQEAKDICEEQVNSLPGSITKAGDFLEANYMNLQRSYTVAIAGYALAQMGRLKGPLLNKFLTTAKDKNRWEDPGKQLYNVEATSYALLALLQLKDFDFVPPVVRWLNEQRYYGGGYGSTQATFMVFQALAQYQKDAPDHQELNLDVSLQLPSR 955 1303 7E2A960D35180388 cfab_human Complement factor B CFB BF BFD C3/C5 convertase Properdin factor B Glycine-rich beta glycoprotein PBF2 protein protein human Homo sapiens Human MGSNLSPQLCLMPFILGLLSGGVTTTPWSLARPQGSCSLEGVEIKGGSFRLLQEGQALEYVCPSGFYPYPVQTRTCRSTGSWSTLKTQDQKTVRKAECRAIHCPRPHDFENGEYWPRSPYYNVSDEISFHCYDGYTLRGSANRTCQVNGRWSGQTAICDNGAGYCSNPGIPIGTRKVGSQYRLEDSVTYHCSRGLTLRGSQRRTCQEGGSWSGTEPSCQDSFMYDTPQEVAEAFLSSLTETIEGVDAEDGHGPGEQQKRKIVLDPSGSMNIYLVLDGSDSIGASNFTGAKKCLVNLIEKVASYGVKPRYGLVTYATYPKIWVKVSEADSSNADWVTKQLNEINYEDHKLKSGTNTKKALQAVYSMMSWPDDVPPEGWNRTRHVIILMTDGLHNMGGDPITVIDEIRDLLYIGKDRKNPREDYLDVYVFGVGPLVNQVNINALASKKDNEQHVFKVKDMENLEDVFYQMIDESQSLSLCGMVWEHRKGTDYHKQPWQAKISVIRPSKGHESCMGAVVSEYFVLTAAHCFTVDDKEHSIKVSVGGEKRDLEIEVVLFHPNYNINGKKEAGIPEFYDYDVALIKLKNKLKYGQTIRPICLPCTEGTTRALRLPPTTTCQQQKEELLPAQDIKALFVSEEEKKLTRKEVYIKNGDKKGSCERDAQYAPGYDKVKDISEVVTPRFLCTGGVSPYADPNTCRGDSGGPLIVHKRSRFIQVGVISWGVVDVCKNQKRQKQVPAHARDFHINLFQVLPWLKEKLQDEDLGFL DIP protein P00751 original sequence version: 153 8BB6C101CC6AC200 fhr1_human Complement factor H-related protein 1 CFHR1 CFHL CFHL1 CFHL1P CFHR1P FHR1 HFL1 HFL2 H factor-like protein 1 H36 protein protein human Homo sapiens Human MWLLVSVILISRISSVGGEATFCDFPKINHGILYDEEKYKPFSQVPTGEVFYYSCEYNFVSPSKSFWTRITCTEEGWSPTPKCLRLCFFPFVENGHSESSGQTHLEGDTVQIICNTGYRLQNNENNISCVERGWSTPPKCRSTDTSCVNPPTVQNAHILSRQMSKYPSGERVRYECRSPYEMFGDEEVMCLNGNWTEPPQCKDSTGKCGPPPPIDNGDITSFPLSVYAPASSVEYQCQNLYQLEGNKRITCRNGQWSEPPKCLHPCVISREIMENYNIALRWTAKQKLYLRTGESAEFVCKRGYRLSSRSHTLRTTCWDGKLEYPTCAKR C0A1B38B8B34B6EF p01024-pro_0000005911 Complement C3b alpha' chain C3 CPAMD1 C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1 protein protein human Homo sapiens Human SNLDEDIIAEENIVSRSEFPESWLWNVEDLKEPPKNGISTKLMNIFLKDSITTWEILAVSMSDKKGICVADPFEVTVMQDFFIDLRLPYSVVRNEQVEIRAVLYNYRQNQELKVRVELLHNPAFCSLATTKRRHQQTVTIPPKSSLSVPYVIVPLKTGLQEVEVKAAVYHHFISDGVRKSLKVVPEGIRMNKTVAVRTLDPERLGREGVQKEDIPPADLSDQVPDTESETRILLQGTPVAQMTEDAVDAERLKHLIVTPSGCGEQNMIGMTPTVIAVHYLDETEQWEKFGLEKRQGALELIKKGYTQQLAFRQPSSAFAAFVKRAPSTWLTAYVVKVFSLAVNLIAIDSQVLCGAVKWLILEKQKPDGVFQEDAPVIHQEMIGGLRNNNEKDMALTAFVLISLQEAKDICEEQVNSLPGSITKAGDFLEANYMNLQRSYTVAIAGYALAQMGRLKGPLLNKFLTTAKDKNRWEDPGKQLYNVEATSYALLALLQLKDFDFVPPVVRWLNEQRYYGGGYGSTQATFMVFQALAQYQKDAPDHQELNLDVSLQLPSRSSKITHRIHWESASLLRSEETKENEGFTVTAEGKGQGTLSVVTMYHAKAKDQLTCNKFDLKVTIKPAPETEKRPQDAKNTMILEICTRYRGDQDATMSILDISMMTGFAPDTDDLKQLANGVDRYISKYELDKAFSDRNTLIIYLDKVSHSEDDCLAFKVHQYFNVELIQPGAVKVYAYYNLEESCTRFYHPEKEDGKLNKLCRDELCRCAEENCFIQKSDDKVTLEERLDKACEPGVDYVYKTRLVKVQLSNDFDEYIMAIEQTIKSGSDEVQVGQQRTFISPIKCREALKLEEKKHYLMWGLSSDFWGEKPNLSYIIGKDTWVEHWPEEDECQDEENQKQCQDLGAFTESMVVFGCPN 749 1663 1E32EA0CEA20EA81 heparin Heparin small molecule small molecule chemical synthesis Chemical synthesis (Chemical synthesis) fhr5_human Complement factor H-related protein 5 CFHR5 CFHL5 FHR5 protein protein human Homo sapiens Human MLLLFSVILISWVSTVGGEGTLCDFPKIHHGFLYDEEDYNPFSQVPTGEVFYYSCEYNFVSPSKSFWTRITCTEEGWSPTPKCLRMCSFPFVKNGHSESSGLIHLEGDTVQIICNTGYSLQNNEKNISCVERGWSTPPICSFTKGECHVPILEANVDAQPKKESYKVGDVLKFSCRKNLIRVGSDSVQCYQFGWSPNFPTCKGQVRSCGPPPQLSNGEVKEIRKEEYGHNEVVEYDCNPNFIINGPKKIQCVDGEWTTLPTCVEQVKTCGYIPELEYGYVQPSVPPYQHGVSVEVNCRNEYAMIGNNMITCINGIWTELPMCVATHQLKRCKIAGVNIKTLLKLSGKEFNHNSRIRYRCSDIFRYRHSVCINGKWNPEVDCTEKREQFCPPPPQIPNAQNMTTTVNYQDGEKVAVLCKENYLLPEAKEIVCKDGRWQSLPRCVESTAYCGPPPSINNGDTTSFPLSVYPPGSTVTYRCQSFYKLQGSVTVTCRNKQWSEPPRCLDPCVVSEENMNKNNIQLKWRNDGKLYAKTGDAVEFQCKFPHKAMISSPPFRAICQEGKFEYPICE 7FAAE31707B0C112 cfhr1-cfhr2-1 2 16 unspecified role unspecified role neutral component neutral component purified purified 13 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 4 A/B, Lanes 12 + 13. Lanes 12 and 13 corresponded to different FHR1-FHR5 hetero-oligomers. cfhr1-cfhr5-1 7 9 unspecified role unspecified role neutral component neutral component partially purified partially purified 13 unspecified role unspecified role neutral component neutral component partially purified partially purified Q03591:p.[His157Tyr;Leu159Val;Glu175Gln] mutation with no effect mutation with no effect mutation with no effect mutation not affecting interaction certain certain sequence position certain certain certain sequence position certain H Y certain certain sequence position certain certain certain sequence position certain L V certain certain sequence position certain certain certain sequence position certain E Q Tue Jun 30 19:36:19 BST 2020 This feature has been corrected as a result of our quality control procedures. The original label was 'p.[His157Tyr;Leu159Val;Glu175Gln]' physical association physical association Figure 3, 5A, Supplementary figure 5. Identical results were obtained with FH-depleted plasma from individuals with each of the 3 CFHR1*A/B genotypes. 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. cfhr1-cfhr5-2 7 16 unspecified role unspecified role neutral component neutral component partially purified partially purified 13 unspecified role unspecified role neutral component neutral component partially purified partially purified Q03591:p.[His157Tyr;Leu159Val;Glu175Gln] mutation with no effect mutation with no effect mutation with no effect mutation not affecting interaction certain certain sequence position certain certain certain sequence position certain H Y certain certain sequence position certain certain certain sequence position certain L V certain certain sequence position certain certain certain sequence position certain E Q Tue Jun 30 18:21:57 BST 2020 This feature has been corrected as a result of our quality control procedures. The original label was 'p.[His157Tyr;Leu159Val;Glu175Gln]' physical association physical association Figure 3, Supplementary figure 5. Identical results were obtained with FH-depleted plasma from individuals with each of the 3 CFHR1*A/B genotypes. cfhr1-cfhr2-2 2 9 unspecified role unspecified role neutral component neutral component purified purified 13 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 4A/B, Lane 7 cfhr1-cfhr2-3 2 13 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 4A/B, Lane 1+2 cfhr1-cfhr5-3 2 16 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 4 A/B, Lane 4 FHR2 and FHR5 only formed homo-oligomers in the absence of FHR1. cfhr5-1 2 9 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 4 A/B, Lane 5 FHR2 and FHR5 only formed homo-oligomers in the absence of FHR1. cfhr1-cfhr2-4 6 13 unspecified role unspecified role neutral component neutral component partially purified partially purified 9 unspecified role unspecified role neutral component neutral component partially purified partially purified physical association physical association Figure 5B, Supp. fig 8. 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. cfhr1-7 5 13 unspecified role unspecified role neutral component neutral component purified purified 13 unspecified role unspecified role neutral component neutral component purified purified direct interaction direct interaction Figure 6 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). it is not stated if the mutant FHR1 is from GN29M which is heterozgous for the deletion or, GN29 which is not. cfhr1-cfhr1-2 1 13 unspecified role unspecified role prey prey purified purified human-plasma homo sapiens blood plasma plasma blood plasma 11 unspecified role unspecified role bait bait purified purified human-plasma homo sapiens blood plasma plasma blood plasma direct interaction direct interaction Figure 7 Mutant FHR1 bound more efficiently to C3dg than the native FHR1 protein. c3-cfhr1-5 1 13 unspecified role unspecified role prey prey purified purified human-plasma homo sapiens blood plasma plasma blood plasma 49 unspecified role unspecified role bait bait purified purified human-plasma homo sapiens blood plasma plasma blood plasma physical association physical association Figure 7 Mutant FHR1 bound more efficiently to C3b and iC3b than the native FHR1 protein. mutant FHR1 bound more efficiently to C3b than the native FHR1 protein. iC3b cannot be entered as an interactor as it's structure is not defined. c3-cfhr1-6 1 10 unspecified role unspecified role prey prey purified purified human-plasma homo sapiens blood plasma plasma blood plasma 49 unspecified role unspecified role bait bait purified purified human-plasma homo sapiens blood plasma plasma blood plasma physical association physical association Figure 7B Conversion to iC3b almost eliminated binding of FH. cfh-heparin-1 3 13 weight by comassie molecular weight estimation by coomasie staining weight by comassie ms participant Identification by mass spectrometry ms participant experimental particp experimental participant identification experimental particp unspecified role unspecified role prey prey physiological level physiological level endogenous level endogenous endogenous endogenous level human-plasma homo sapiens blood plasma plasma blood plasma 10 weight by comassie molecular weight estimation by coomasie staining weight by comassie ms participant Identification by mass spectrometry ms participant experimental particp experimental participant identification experimental particp unspecified role unspecified role prey prey physiological level physiological level endogenous level endogenous endogenous endogenous level human-plasma homo sapiens blood plasma plasma blood plasma 15 predetermined predetermined participant predetermined experimental particp experimental participant identification experimental particp unspecified role unspecified role bait bait purified purified association association Supplementary Figure 2 Plasma from patient GN29 with mutant FHR1 which has an internal duplication of CFHR1 in the genomic DNA , encompassing exons 2–5. I also put "Figure 1C" here - which was obviously wrong - but I cannot find out which figure I meant- if any, so just left Supp. fig 2 here. cfhr1-cfhr2-5 6 13 unspecified role unspecified role neutral component neutral component partially purified partially purified 16 unspecified role unspecified role neutral component neutral component partially purified partially purified physical association physical association Supp. fig 8. Results obtained from normal plasma. c3-cfh-11 1 12 unspecified role unspecified role prey prey purified purified human-plasma homo sapiens blood plasma plasma blood plasma 49 unspecified role unspecified role bait bait purified purified human-plasma homo sapiens blood plasma plasma blood plasma physical association physical association Supplementary Figure 9 AP proconvertase was formed. i have not attempted to add more SPR results from Supp. figs 9 +10 as it is not clear what is reacting with what. c3b_human Complement C3b complex C3b dimer 14 unspecified role unspecified role C3 beta chain binding region binding region binding-associated region binding region undetermined undetermined sequence position undetermined undetermined undetermined sequence position undetermined 8 unspecified role unspecified role C3b alpha(prime) chain binding region binding region binding-associated region binding region undetermined undetermined sequence position undetermined undetermined undetermined sequence position undetermined 66 64 physical association physical association human Homo sapiens Human stable complex stable complex physical interaction evidence used in manual assertion physical interaction evidence used in manual assertion IPI inferred from physical interaction 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. 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. Heterodimer 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. 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. 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. Accepted 2017-JAN-19 by ORCHARD coronavirus