SRP6317

C1 Esterase inhibitor from human plasma

≥95% (SDS-PAGE)

• Synonym(e): C1-inhibiting factor, Complement C1 esterase inhibitor, Esterase inhibitor C-1, Plasma protease C1 inhibitor, Serpin G1
• NACRES: NA.32
• UNSPSC Code: 12352204
• Biological source: human
• Assay: ≥95% (SDS-PAGE)
• Form: frozen liquid
• Mol wt: 100 kDa

Eigenschaften

• biological source: human
• assay: ≥95% (SDS-PAGE)
• form: frozen liquid
• mol wt: 100 kDa
• packaging: pkg of 1 mg
• UniProt accession no.: P05155
• shipped in: dry ice
• storage temp.: −70°C
• Gene Information: human ... Serpin G1(710)

Beschreibung

General description

C1 esterase inhibitor is a single chain glycoprotein which inhibits C1, C1r, C1s, plasma kallikrein, factors XIa, XIIa and plasmin of the blood clotting system. It is present in the plasma at 16-33 mg/100mL. It is part of the serpin family.

Biochem/physiol Actions

C1 esterase inhibitor functions as a serine proteinase inhibitor. 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. 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 protein is non-functional. C1 esterase inhibitor deficiencies also disturb the fibrinolytic system, the intrinsic coagulation pathway and the complement pathway.

Physical form

Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.

Sicherheitshinweise

• Lagerklasse: 11 - Combustible Solids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable