C1 Esterase inhibitor Protein | SERPING1 protein

C1 Esterase inhibitor, Human Plasma

Cat. Num. AAA844047

• Gene Names: SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH
• Applications: SDS-Page
• Purity: >=95%
• Synonyms: C1 Esterase inhibitor; Human Plasma; Plasma protease C1 inhibitor; C1-inhibiting factor; Serpin G1; Complement C1 esterase inhibitor; Esterase inhibitor C-1; SERPING1 protein

• Host: Human Plasma
• Purity/Purification: >=95%
• Form/Format: Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.; Appearance: Frozen
• Sequence Length: 500

• Applicable Applications for SERPING1 protein: SDS-PAGE
• Extinction Coefficient: 0.4
• Handling: Centrifuge the vial prior to opening.
• Dry Ice Shipment: Extra charge fee may add to your shipping cost as dry ice is required to ship this product.
• Preparation and Storage: At -80 degree C; Avoid repeated freezing and thawing cycles.

Testing Data

Related Product Information for SERPING1 protein

Background: A single chain glycoprotein which inhibits C1, C1r, C1s, plasma kallikrein, Factors XIa. XIIa, and plasmin. Present in plasma at 16-33 mg per 100 ml. C1 esterase inhibitor deficiency is a rare condition resulting in facial swelling and abdominal cramping. Usually the condition is hereditary, though it may also occur when the C1EI is non-functional. The C1 esterase inhibitor protein is a normal constituent of serum which functions as a serine proteinase inhibitor of the serpin family. The C1 esterase inhibitor inhibits the complement proteases C1r and C1s, as well as the proteases kallikrein, factor XIa, XIIa and plasmin of the blood clotting system. The concentration of C1 esterase inhibitor protein is reduced to 10-30% of normal in patients with angioedema secondary to C1 esterase inhibitor deficiency (85% of patients with Hereditary Angioedema (HAE)); in 15% of patients with HAE, the concentrations of the inhibitor protein is normal but function is markedly reduced.

Product Categories/Family for SERPING1 protein

Proteins and Enzymes; Native Proteins (NEW CATEGORY) Proteins and Enzymes; Proteins and Enzymes (A-Z)

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; A6NMU0; A8KAI9; B2R6L5; B4E1F0; B4E1H2
• Molecular Weight: 100 kDa
• 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
• 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: Angioedema, Hereditary, Type I; Complement Component 4, Partial Deficiency Of