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, i.e., a 5 strand β-sandwich arrangement).[1]

Disease

CFH mutations are involved in dense deposit disease, age-related macular degeneration, atypical hemolytic-uremic syndrome, schizophrenia, stroke and the kidney disorder glomerulonephritis.

Relevance

CFH is recruited by human pathogens resulting in increased virulence.

Structural highlights

The sushi domains are ca. 60 amino acid long containing 2 Cys-Cys bonds.


Structure of human complement factor H 19-20 sushi domains (magenta) complex with complement C3 (green) and glycerol (PDB code 3oxu).

Drag the structure with the mouse to rotate

3D structures of complement factor H3D structures of complement factor H

Updated on 20-December-2015



ReferencesReferences

  1. Rodriguez de Cordoba S, Esparza-Gordillo J, Goicoechea de Jorge E, Lopez-Trascasa M, Sanchez-Corral P. The human complement factor H: functional roles, genetic variations and disease associations. Mol Immunol. 2004 Jun;41(4):355-67. PMID:15163532 doi:10.1016/j.molimm.2004.02.005

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