Recombinant Human Complement factor H (CFH), Truncated

Uniprot Id

P08603

Target Species

Human

Target Name

CFH

Target Full Name

Complement factor H

Target Function

Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces. Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of the complement amplification loop. As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b. In addition, mediates several cellular responses through interaction with specific receptors. For example, interacts with CR3/ITGAM receptor and thereby mediates the adhesion of human neutrophils to different pathogens. In turn, these pathogens are phagocytosed and destroyed.

Target Involvement

Basal laminar drusen (BLD); Complement factor H deficiency (CFHD); Hemolytic uremic syndrome atypical 1 (AHUS1); Macular degeneration, age-related, 4 (ARMD4)

Target Subcellular Location

Secreted.

Target Tissue Specificity

Expressed in the retinal pigment epithelium (at protein level). CFH is one of the most abundant complement components in blood where the liver is the major source of CFH protein in vivo. in addition, CFH is secreted by additional cell types including mono

Target Research Area

Cancer

Target Synonyms

adrenomedullin binding protein; age related maculopathy susceptibility 1; AHUS 1; AHUS1; AMBP 1; AMBP1; ARMD 4; ARMD4; ARMS 1; ARMS1; beta 1 H globulin; beta 1H; beta1H; CFAH_HUMAN; CFH; CFHL 3; CFHL3; Complement factor H; complement factor H; isoform b; Factor H; factor H like 1; FH; FHL 1; FHL1; H factor 1 (complement); H factor 1; H factor 2 (complement); HF 1; HF 2; HF; HF1; HF2; HUS; MGC88246

Target Background

This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.