C2 Background Information
The complement component proteins: C2, C3, C4, and C5 are potent anaphylatoxins that are released during complement activation. Binding of these proteins to their respective G protein-coupled receptors induces proinflammatory events such as cellular degranulation, smooth muscle contraction, arachidonic acid metabolism, cytokine release, leukocyte activation, and cellular chemotaxis. C2 deficiency (C2D) is the most common deficiency of the classical complement pathway and is mostly found in patients with autoimmune disease or susceptibility to bacterial infections. The N-terminal extracellular domain 1 of complement C2 receptor inhibitory trispanning, or CRIT, binds to C2 and specifically interacts with the C2a fragment. In doing so, CRIT blocks C2 cleavage and also prevents the classical pathway of C3 convertase formation.
