Factor H Recombinant Rabbit Monoclonal Antibody [JA52-13] (ET1704-83)

UNIPROT #

P08603

PROTEIN NAME

Factor H

SYNONYMS

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

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 monocytes, fibroblasts, or endothelial cells.

POST-TRANSLATIONAL MODIFICATION

Sulfated on tyrosine residues.

SUBCELLULAR LOCATION

Secreted.

FUNCTION

The Factor H gene family is a multidomain, multifunctional protein family whose individual members are defined by conserved structural elements, which display diverse yet often overlapping functions. These proteins share a common structural motif, the short consensus repeat (SCR), which is structurally conserved among related genes and between phylogenetically divergent species. The human complement Factor H (FH, CFH, HUS, b-1H) gene encodes a 1213 amino acid serum glycoprotein which is arranged into 20 SCRs, each approximately 60 amino acids long, and an 18-residue leader sequence. Factor H controls the function of the alternative complement pathway and acts as a cofactor with Factor I (C3b inactivator). In addition, Factor H has functional activity outside of the complement system, where it can bind to the cellular integrin receptor (CD11b/CD18), interact with cell surface glycosaminoglycans and associate with the surface of certain pathogenic microorganisms. Deficiencies in Factor H is a common characteristic of acute renal disease.