SERPING1
SERPING1 (serpin family G member 1) encodes plasma protease C1 inhibitor, a secreted serine protease inhibitor that regulates the classical complement pathway. It is also known as C1-INH, C1INH, C1IN, C1NH, HAE1 and HAE2. Browse Assay Genie's current range of SERPING1 research reagents below, or read on for the biology behind the target.
SERPING1 encodes plasma protease C1 inhibitor, a serine protease inhibitor that acts as a regulator of the classical complement pathway. It forms a proteolytically inactive, stoichiometric complex with the C1r or C1s proteases, so inhibiting activated C1r and C1s of the first complement component and limiting complement activation. The protein may also regulate blood coagulation, fibrinolysis and the generation of kinins. It is a very efficient inhibitor of FXIIa, and it also inhibits chymotrypsin and kallikrein.
The SERPING1 gene encodes a highly glycosylated plasma protein that is synthesised in the liver and secreted. Alternative splicing produces multiple transcript variants, all of which encode the same isoform. The protein belongs to the serpin family, and HGNC places the gene in the groups of serpin peptidase inhibitors and complement system regulators and receptors. Mouse and rat orthologs, both named Serping1, are recognised.
Deficiency of C1 inhibitor is associated with hereditary angioneurotic oedema (HANE), which explains the aliases HAE1 and HAE2 and the earlier gene name serpin peptidase inhibitor, clade G (C1 inhibitor), member 1, (angioedema, hereditary). Researchers studying complement regulation, contact-system proteases or angioedema commonly work with C1-INH, whether through a SERPING1 antibody, a C1 inhibitor ELISA kit or the recombinant SERPING1 protein.
C1 inhibitor forms inactive complexes with C1r and C1s, making it a central control point in the classical complement pathway.
Deficiency of this protein is associated with hereditary angioneurotic oedema, linking SERPING1 to the HAE1 and HAE2 designations.
C1 inhibitor may regulate blood coagulation and fibrinolysis, and it is a very efficient inhibitor of FXIIa.
The protein may influence kinin generation and inhibits kallikrein and chymotrypsin, which makes it relevant to protease inhibition studies.
SERPING1 encodes C1 inhibitor, a serine protease inhibitor that regulates the classical complement pathway. It forms a proteolytically inactive complex with C1r or C1s. It may also regulate coagulation, fibrinolysis and kinin generation, and it inhibits FXIIa, kallikrein and chymotrypsin.
The protein is called plasma protease C1 inhibitor, with aliases C1-INH, C1INH, C1IN, C1NH, HAE1 and HAE2. Earlier gene names include serine (or cysteine) proteinase inhibitor, clade G (C1 inhibitor), member 1. Alternative splicing gives several transcripts that encode the same isoform.
C1 inhibitor deficiency is associated with hereditary angioneurotic oedema. The gene has mouse and rat orthologs, both named Serping1. Check the datasheet of any product for its validated reactivity.
Other complement components, serpin family members and contact-system proteases that act alongside C1 inhibitor.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P05155; product specifications from Assay Genie datasheets.