Function of the C1 inhibitor

C1 inhibitor (a serpin or serine protease inhibitor) regulates the activation of the classical complement pathway by binding to and inactivating the serine protease subcomponents of C1 (ie, the tetramer consisting of two C1r and two C1s proteins). In this process, the C1 inhibitor complex dissociates from the C1q subcomponent of C1, which remains bound to the immune complex. Note that C1 inhibitor also regulates in the same manner the mannose-binding lectin-associated serine proteases (MASPs) that are the C1r/C1s equivalents in the lectin pathway of complement activation.

Ab: antibody; Ag: antigen.

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Function of the C1 inhibitor