C8B Antibody – Rabbit Polyclonal

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]

C8B: Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. Defects in C8B are a cause of complement component 8 deficiency type 2 (C8D2). A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. Belongs to the complement C6/C7/C8/C9 family.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 1p32.2

Cellular Component: membrane attack complex; extracellular space; membrane; extracellular region; vesicle

Molecular Function: protein complex binding

Biological Process: cytolysis; complement activation, alternative pathway; regulation of complement activation; innate immune response; immune response; complement activation, classical pathway; complement activation

Disease: Complement Component 8 Deficiency, Type Ii