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complement factor H (CFH)
Function:
- binds to C3b enhancing conversion of C3b to iC3b (inactive form) by Factor I
- also increases the rate of dissociation of the C3bBb complex (C3 convertase) & the (C3b)NBB complex (C5 convertase) in the alternative complement pathway
- binds to heparin & C-reactive protein [4]
Structure:
- each Sushi domain binds C1 esterase inhibitor
Expression: expressed by the liver & secreted into plasma
Polymorphism:
- polymorphism associated with macular degeneration
Interactions
molecular events
Related
CFH gene mutation
complement cascade
complement factor H-related protein 1; FHR-1; H factor-like protein 1; H-factor-like 1; H36 (CFHR1, CFHL, CFHL1, CFHL1P, CFHR1P, FHR1, HFL1, HFL2)
complement factor H-related protein 2; FHR-2; DDESK59; H factor-like 3; H factor-like protein 2 (CFHR2 CFHL2 FHR2 HFL3)
complement factor H-related protein 3; FHR-3; DOWN16; H factor-like protein 3 (CFHR3, CFHL3, FHR3)
General
complement
Properties
SIZE: MW = 139 kD
entity length = 1231 aa
COMPARTMENT: plasma
MOTIF: signal sequence {1-18}
Sushi domain {19-1230} (20)
MOTIF: cysteine residue {C21}
MODIFICATION: cysteine residue {C66}
cysteine residue {C52}
MODIFICATION: cysteine residue {C80}
cysteine residue {C66}
MODIFICATION: cysteine residue {C21}
cysteine residue {C80}
MODIFICATION: cysteine residue {C52}
N-glycosylation site {N1095}
Database Correlations
OMIM 134370
MORBIDMAP 134370
UniProt P08603
References
- Baron M, Norman DG, Campbell ID.
Protein modules.
Trends Biochem Sci. 1991 Jan;16(1):13-7. Review.
PMID: 2053133
- Clinical Diagnosis and Management by Laboratory
Methods, 18th ed, J.B. Henry (ed), W.B. Saunders,
Philadelphia, PA, 1991 pg 835
- OMIM :accession 134370
- Klein RJ, Zeiss C, Chew EY, Tsai JY, Sackler RS, Haynes C,
Henning AK, Sangiovanni JP, Mane SM, Mayne ST, Bracken MB,
Ferris FL, Ott J, Barnstable C, Hoh J.
Complement factor H polymorphism in age-related macular
degeneration.
Science. 2005 Apr 15;308(5720):385-9. Epub 2005 Mar 10.
PMID: 15761122