Entrez Summary: This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. This protein co-localizes with C3, binds C3b in a dose-dependent manner, and is recruited to tissues damaged by C-reactive protein. Allelic variations in this gene have been associated, but not causally linked, with two different forms of kidney disease: membranoproliferative glomerulonephritis type II (MPGNII) and hemolytic uraemic syndrome (HUS). [provided by RefSeq, Jan 2010].
UniProt Summary: Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. {ECO:0000269|PubMed:23487775}.
Pfam DomainsGO Terms
Pfam Domains
Sushi
GO Terms
positive regulation of cytolysis
complement activation, alternative pathway
regulation of cytolysis
negative regulation of protein binding
regulation of complement activation
regulation of humoral immune response
negative regulation of binding
complement activation
regulation of protein binding
humoral immune response
regulation of binding
regulation of immune effector process
protein heterodimerization activity
protein-containing complex
activation of immune response
innate immune response
positive regulation of immune response
protein homodimerization activity
defense response to other organism
immune effector process
negative regulation of molecular function
regulation of immune response
positive regulation of immune system process
response to other organism
response to external biotic stimulus
response to biotic stimulus
defense response
regulation of immune system process
immune response
extracellular region
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)