Function
Complement factor H (CFH) regulates the complement system which is part of the immune response. CFH protects cells by preventing the activation of the complement system when it is not needed. CFH contains 20 short consensus repeats (sushi domains).
Disease
CFH mutations are involved in dense deposit disease, age-related macular degeneration, atypical hemolytic-uremic syndrome and the kidney disorder glomerulonephritis.
Relevance
Structural highlights
3D structures of complement factor H
Updated on 26-August-2014
Complement factor H
1haq, 3gau, 3gav, 3gaw – hCFH – human - NMR
2rlp – hCFH 1-2 sushi domains - NMR
2rlq – hCFH 2-3 sushi domains - NMR
2qfg – hCFH 1-5 sushi domains
2yby – mCFH 6-8 sushi domains (mutant) - mouse
2ic4 – hCFH 6-8 sushi domains (mutant)
2jgw – hCFH 7 sushi domain – NMR
2jgx – hCFH 7 sushi domain (mutant)
4b2r – hCFH 10-11 sushi domains - NMR
4b2s – hCFH 11-12 sushi domains - NMR
2kms – hCFH 12-13 sushi domains – NMR
1hfi – hCFH 15 sushi domain – NMR
1hfh – hCFH 15-16 sushi domains – NMR
2qfh – hCFH 16-20 sushi domains - NMR
3sw0 – hCFH 18-20 sushi domains
2bzm – hCFH 19-20 sushi domains – NMR
3kzj – hCFH 19-20 sushi domains (mutant) – NMR
2g7i – hCFH 19-20 sushi domains
3kxv, 3r62 – hCFH 19-20 sushi domains (mutant)
Complement factor H complexes
2wii – hCFH 1-4 sushi domains + complement C3
2w80, 2w81, 4ayd, 4aye, 4ayi, 4aym – hCFH 6-7 sushi domains + factor H binding protein
2uwn, 2v8e – hCFH 6-8 sushi domains + sucrose octasulfate
4k12 – hCFH 9 sushi domain + choline binding protein A
3oxu – hCFH 19-20 sushi domains + complement C3
2xqw – hCFH 19-20 sushi domains (mutant) + complement C3
4j38 – hCFH 19-20 sushi domains (mutant) + outer surface protein E
4k12 – hCFH 9 sushi domain + choline binding protein A
References
