Recombinant Human Plasma protease C1 inhibitor (SERPING1)

Uniprot Id

P05155

Target Species

Human

Target Name

SERPING1

Target Full Name

Plasma protease C1 inhibitor

Target Function

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.

Target Involvement

Hereditary angioedema (HAE)

Target Subcellular Location

Secreted.

Target Protein Families

Serpin family

Target Synonyms

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

Target Background

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.