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Product Description
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.
Biovision | 7299 | C1 Esterase inhibitor Human Plasma DataSheet
Biomolecule/Target: C1 Esterase inhibitor
Synonyms: Plasma protease C1 inhibitor, C1-inhibiting factor, Serpin G1, Complement C1 esterase inhibitor, Esterase inhibitor C-1
Alternates names: Plasma protease C1 inhibitor, C1-inhibiting factor, Serpin G1, Complement C1 esterase inhibitor, Esterase inhibitor C-1
Taglines: A serine proteinase inhibitor of the serpin family.
NCBI Gene ID #: 12367
NCBI Gene Symbol: CASP3
Gene Source: Human
Accession #: Q8BNT4
Recombinant: No
Source: Human Plasma
Purity by SDS-PAGEs: N/A
Assay: SDS-PAGE
Purity: N/A
Assay #2: N/A
Endotoxin Level: N/A
Activity (Specifications/test method): N/A
Biological activity: N/A
Results: N/A
Binding Capacity: N/A
Unit Definition: N/A
Molecular Weight: 100 kDa
Concentration: N/A
Appearance: Frozen
Physical form description: Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.
Reconstitution Instructions: N/A
Amino acid sequence: N/A
