Complement Factor H Products

Description

The complement Factor H protein is secreted into the bloodstream and acts in the regulation of complement activation. Mutations leading to changes in this protein have been linked with HUS (hemolytic-uremic syndrome) and chronic hypocomplementemic nephropathy. Factor H is mainly synthesised in the liver but also in macrophages and endothelium. It is primarily aplasma glycoprotein but is also found in platelets and there is a membrane bound form on some leukocytes. Consisting of a single polypeptide, the major form of Factor H has a molecular weight of 155kDa. There are two truncated forms, a non-glycosylated 49 kDa form and a glycosylated 39-43 kDaform. Plasma concentrations are in the range 200-600mg/L for the 155 kDa form and 1-5mg/L for thetruncated forms. Factor H is a major regulatory protein of the complement system. By binding to C3b it either displacesor prevents the binding of Bb (activated Factor B). When bound to Factor H, C3b is susceptible tocleavage by Factor 1 to yield iC3b. Factor H is released or modified following this cleavage. The regulatory role of Factor H is essential because C3bBb is not only a C5 convertase but a C3 convertaseand so has a positive feedback effect, potentially consuming the entire C3 pool if unregulated.

Bioinformatics

Entrez	12628 Mouse 155012 Rat 3075 Human
Uniprot	AAH20687 Human AAH37285 Human AAH37285.1 Human NM_001014975 Human NP_000177.2 Human NP_001014975.1 Human P08603 Human
Product By Gene ID	3075
Alternate Names	adrenomedullin binding protein age-related maculopathy susceptibility 1 AHUS1 AMBP1 ARMD4 beta-1H beta-1-H-globulin CFHL3 complement factor H factor H factor H-like 1 FH FHL1 H factor 1 (complement) H factor 1 H factor 2 (complement) HF HF1ARMS1 HF2 HUSMGC88246