C8 β Rabbit Polyclonal Antibody

This gene encodes one of the three subunits of the complement component 8 (C8) protein. C8 is composed of equimolar amounts of alpha, beta and gamma subunits, which are encoded by three separate genes. C8 is one component of the membrane attack complex, which mediates cell lysis, and it initiates membrane penetration of the complex. This protein mediates the interaction of C8 with the C5b-7 membrane attack complex precursor. In humans deficiency of this protein is associated with increased risk of meningococcal infections. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2013], disease:Defects in C8B are a cause of complement C8 deficiency type II [MIM:120960]. Patients with deficiency of C8 suffer from recurrent bacterial infections, predominantly from Neisseria meningitidis., function:C8 is a constituent of the membrane attack complex. C8 binds to the C5b-7 complex, forming the C5b-8 complex. C5-b8 binds C9 and acts as a catalyst in the polymerization of C9., online information:C8B mutation db, polymorphism:The sequence shown is that of allotype C8B B., PTM:N-glycosylated; contains one or two bound glycans. Not O-glycosylated., similarity:Belongs to the complement C6/C7/C8/C9 family., similarity:Contains 1 EGF-like domain., similarity:Contains 1 LDL-receptor class A domain., similarity:Contains 1 MACPF domain., similarity:Contains 2 TSP type-1 domains., subunit:C8 is composed of three chains: alpha, beta and gamma. The beta chain binds to the C8 alpha chain and to the C5b-C7 complex, presumably to C5b. It is essential to the incorporation of C8 into the C5b-C8 complex.