Plasma Protease C1 Inhibitor (SERPING1) Reagents

Plasma protease C1 inhibitor

Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.

Hereditary angioedema (HAE)

Secreted.

Serpin family

C1 esterase inhibitor; C1 Inh; C1 inhibiting factor; C1 inhibitor; C1-inhibiting factor; C1IN; C1Inh; C1NH; complement component 1 inhibitor; esterase inhibitor; HAE1; HAE2; IC1_HUMAN; Plasma protease C1 inhibitor; Serine (or cysteine) proteinase inhibitor clade G member 1; serine/cysteine proteinase inhibitor clade G member 1; Serpin G1; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1; SERPING1

This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its encoded protein, C1 inhibitor, inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. It is synthesized in the liver, and its deficiency is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform.