Serpin family G member 1 (SERPING1)

Serpin family G member 1 (SERPING1) encodes C1 inhibitor (C1-INH), a highly glycosylated plasma protein belonging to the serpin superfamily. C1-INH is a critical regulator of the complement system, modulating classical pathway activation by inhibiting proteases such as C1r, C1s, plasma kallikrein, and activated factor XII (factor XIIa). This inhibition limits inflammation, vascular permeability, and edema formation. Mutations in SERPING1 cause hereditary angioedema, characterized by episodic swelling due to uncontrolled bradykinin production. C1-INH is targeted therapeutically in hereditary angioedema and investigated as a biomarker in cardiovascular and inflammatory conditions

Other names

C1 inhibitorC1-INHplasma protease C1 inhibitorserpine1

Replacement therapy: replenishes functional C1 inhibitor to restore regulation of complement and kallikrein/bradykinin pathways. Inhibition of bradykinin-mediated vascular leakage. Inhibition of plasma kallikrein/factor XIIa activity via irreversible binding ("suicide substrate" mechanism).