Serpin Peptidase Inhibitor, Clade G Member 1 Protein | SERPING1 protein

Human Serpin Peptidase Inhibitor, Clade G Member 1

Cat. Num. AAA147407

• Gene Names: SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH
• Purity: Greater than 95.0% as determined by SDS-PAGE.
• Synonyms: Serpin Peptidase Inhibitor; Clade G Member 1; Human Serpin Peptidase Inhibitor; Clade G Member 1 Human; C1IN; C1INH; C1NH; HAE1; HAE2; Plasma protease C1 inhibitor; C1 esterase inhibitor; C1-inhibiting factor; Serpin G1; Name; SERPING1; SERPING1 Human; Native; SERPING1 Native; SERPING1 protein

• Host: Human Plasma
• Purity/Purification: Greater than 95.0% as determined by SDS-PAGE.
• Form/Format: 10mM sodium phosphate and 145mM NaCl, pH7.3.; Sterile Filtered solution.

• Human Virus Test: Plasma from each donor has been tested and found negative for antibody to HIV-1, HIV-2, HCV, HTLV-I &II, STS and HBSAG.
• Preparation and Storage: Native SERPING1 Human is stable at 4 degree C if entire vial will be used within 2-4 weeks.; Store, frozen below -20 degree C for longer periods of time.; For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).; Avoid multiple freeze-thaw cycles.

Related Product Information for SERPING1 protein

Introduction: Plasma protease C1 inhibitor (SERPING1) is a part of the serpin superfamily of serine protease inhibitors. SERPING1 plays an important role in regulating activation of both the complement and contact systems. SERPING1 is also activating C1 by coupling with the active catalytic site at the light chains of C1r and C1s. SERPING1 insufficiency results in hereditary angioedema, which is characterized by recurrent episodes of localized angioedema of the skin, gastrointestinal mucosa or upper respiratory mucosa.
Description: Human Serpin Peptidase Inhibitor, Clade G Member 1 produced in Human plasma having a molecular mass of 110kDa.

Product Categories/Family for SERPING1 protein

RECOMBINANT & NATURAL PROTEINS

NCBI and Uniprot Product Information

• NCBI GI #: 73858568
• NCBI GeneID: 710
• NCBI Accession #: NP_000053.2
• NCBI GenBank Nucleotide #: NM_000062.2
• UniProt Accession #: P05155; Q16304; Q547W3; Q59EI5; Q7Z455; Q96FE0; Q9UC49; Q9UCF9; A6NMU0; A8KAI9; B2R6L5
• Molecular Weight: 55,154 Da
• NCBI Official Full Name: plasma protease C1 inhibitor
• NCBI Official Synonym Full Names: serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
• NCBI Official Symbol: SERPING1
• NCBI Official Synonym Symbols: C1IN; C1NH; HAE1; HAE2; C1INH
• NCBI Protein Information: plasma protease C1 inhibitor; serpin G1; C1-inhibiting factor; C1 esterase inhibitor; complement component 1 inhibitor; serine/cysteine proteinase inhibitor clade G member 1
• UniProt Protein Name: Plasma protease C1 inhibitor
• Protein Family: Plasma protease C1 inhibitor
• UniProt Gene Name: SERPING1
• UniProt Synonym Gene Names: C1IN; C1NH; C1 Inh; C1Inh
• UniProt Entry Name: IC1_HUMAN

NCBI Description

This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its protein inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. Deficiency of this protein is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform. [provided by RefSeq, Jul 2008]

Uniprot Description

SERPING1: a protein protease inhibitor (C1-inhibitor) that forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play an important role in regulating complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Mutations of the SERPING1 gene is associated with adult macular degeneration can also cause hereditary angioedema. Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis. Belongs to the serpin family.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 11q12.1

Cellular Component: extracellular space; extracellular region

Molecular Function: serine-type endopeptidase inhibitor activity; protein binding

Biological Process: negative regulation of complement activation, lectin pathway; platelet activation; fibrinolysis; platelet degranulation; blood circulation; innate immune response; blood coagulation; blood coagulation, intrinsic pathway; complement activation, classical pathway; aging

Disease: Complement Component 4, Partial Deficiency Of; Angioedema, Hereditary, Type I