A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a serine PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of complement c1q to antibodies, the activated complement c1r cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves complement c2 and complement c4 to form C4b2a (CLASSICAL C3 CONVERTASE).

Symptoms and diagnosis




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