Complement Factor B is a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb is a serine protease that associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. Complement Factor B is linked to major histocompatibility complex (MHC) class III and is involved in the regulation of the immune reaction. Mutations in Complement Factor B are associated with a reduced risk of age-related macular degeneration (1).
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