Goat C1 Inhibitor Polyclonal Antibody | anti-SERPING1 antibody

Goat anti-human C1 Esterase Inhibitor, Purified IgG

Cat. Num. AAA512026

• Gene Names: SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH
• Purity: Purified IgG
• Synonyms: C1 Inhibitor; Polyclonal Antibody; Goat anti-human C1 Esterase Inhibitor; Purified IgG; human; anti-SERPING1 antibody

• Host: Goat
• Clonality: Polyclonal
• Specificity: This antibody is specific for C1 esterase inhibitor as demonstrated by immunoelectrophoresis and ELISA.
• Purity/Purification: Purified IgG

• Application Notes: This product is suitable as a source of antibodies to C1 esterase inhibitor.
• Immunogen: C1 esterase inhibitor (also called C1-Inactivator) purified from human plasma.
• Presentation: XXXX ml of immunoglobulin purified from goat antiserum. The IgG concentration is XXXX mg/ml in 10 mM HEPES, pH 7.4, containing 0.15 M NaCl and 50% glycerol (v/v). Total protein is 5 mg.
• Preparation and Storage: Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use.

NCBI and Uniprot Product Information

• NCBI GI #: 1197499
• NCBI GeneID: 710
• NCBI Accession #: CAA30314.1
• NCBI GenBank Nucleotide #: X07427.1
• UniProt Accession #: P05155; Q16304; Q547W3; Q59EI5; Q7Z455; Q96FE0; Q9UC49; Q9UCF9; A6NMU0; A8KAI9; B2R6L5; Q5UGI5; Q5UGI6
• Molecular Weight: 55,154 Da
• NCBI Official Full Name: 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; OTTHUMP00000197003; OTTHUMP00000197004; OTTHUMP00000197007; OTTHUMP00000235286; OTTHUMP00000235287; 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
• 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]

Uniprot Description

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. Ref.21

Subunit structure: Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis. Interacts with MASP1. Ref.22 Ref.23 Ref.25

Subcellular location: Secreted.

Post-translational modification: Highly glycosylated (49%) with N- and O-glycosylation. Ref.2 Ref.24 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31Can be proteolytically cleaved by E.coli stcE. Ref.23 Ref.25

Polymorphism: Chymotrypsin uses Ala-465 as its reactive site in normal plasma protease C1 inhibitor, and His-466 as its reactive site in the variant His-466.

Involvement in disease: Defects in SERPING1 are the cause of hereditary angioedema (HAE) [

MIM:106100]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional. Ref.14 Ref.20 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45

Sequence similarities: Belongs to the serpin family.

Sequence caution: The sequence AAA53096.1 differs from that shown. Reason: Erroneous gene model prediction.