Goat anti-Human C1 Esterase Inhibitor Polyclonal Antibody | anti-SERPING1 antibody

C1 Esterase Inhibitor (C1-INH)

Cat. Num. AAA627914

• Gene Names: SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH
• Reactivity: Human
• Applications: ELISA
• Purity: Highly Purified; Purified by ion exchange chromatography.
• Synonyms: C1 Esterase Inhibitor; Polyclonal Antibody; C1 Esterase Inhibitor (C1-INH); Anti -C1 Esterase Inhibitor (C1-INH); anti-SERPING1 antibody

• Host: Goat
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human C1 esterase inhibitor.
• Purity/Purification: Highly Purified; Purified by ion exchange chromatography.
• Form/Format: Supplied as a liquid in 0.01M HEPES, pH7.4, 0.15M sodium chloride and 50% glycerol.

• Applicable Applications for anti-SERPING1 antibody: ELISA (EL/EIA)
• Application Notes: Suitable for use in ELISA and Immunoelectrophoresis.
• Immunogen: C1 esterase Inhibitor (also called C1-Inactivator) purified from human plasma.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Related Product Information for anti-SERPING1 antibody

C1-INH is a member of the serpin family of proteases, as are alpha-antitrypsin, antithrombin III, and angiotensinogen. These proteins stoichiometrically inactivate their target proteases by forming stable, one-to-one complexes with the protein to be inhibited. Although synthesized primarily by hepatocytes, C1-INH is also synthesized by monocytes. The regulation of the protein production is not completely understood, but, since patients respond clinically to androgen therapy serum levels of C1-INH increase, it is believed that androgens may stimulate C1-INH synthesis. All C1-INH deficient patients are heterozygous; half the normal level of C1-INH is not believed sufficient to prevent attacks. Although named for its action on the first component of complement (C1 esterase), C1-INH also inhibits components of the fibrinolytic, clotting, and kinin pathways. Specifically, C1-INH inactivates plasmin-activated Hageman factor (factor XII), activated factor XI, PTA, and kallikrein. Within the complement system, C1-INH blocks the activation of C1 and the rest of the classic complement pathway by binding to C1r and C1s. Without C1-INH, unchecked activation of C1, C2, and C4 occur before other inhibitors (C4-binding protein and factor I) can halt the cascade. The actual factor or factors responsible for the edema formation remain somewhat controversial. Researchers have demonstrated activation of the kinin system and increased bradykinin concentration associated with clinical flares. Bradykinin is an important inflammatory mediator that causes neutrophil chemotaxis, capillary dilation, and smooth muscle relaxation, and it has been linked to other forms of angioedema. In an animal model of C1-INH deficiency, bradykinin antagonists prevent capillary leakage. Others implicate C2 kinin, a metabolite of C2b, as the active agent in the presence of plasmin.

Product Categories/Family for anti-SERPING1 antibody

Antibodies; Abs to Protease Inhibitors

NCBI and Uniprot Product Information

• NCBI GI #: 20385946
• NCBI GeneID: 710
• UniProt Accession #: P05155; Q16304; Q547W3; Q59EI5; Q7Z455; Q96FE0; Q9UC49; Q9UCF9; A6NMU0; A8KAI9; B2R6L5
• Molecular Weight: 55,154 Da
• NCBI Official Full Name: C1 esterase 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; complement activation, classical pathway; blood coagulation, intrinsic pathway; aging

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