<StructureSection load='3oxu' size='340' side='right' caption='Structure of human complement factor H 19-20 sushi domains (yellow, magenta, cyan) complex with complement C3 (grey, green, pink) and glycerol (PDB code 3oxu).' scene=>
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 20-November-2014
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- 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
