SRP6317

C1 Esterase inhibitor from human plasma

≥95% (SDS-PAGE)

• 同義詞: C1-inhibiting factor, Complement C1 esterase inhibitor, Esterase inhibitor C-1, Plasma protease C1 inhibitor, Serpin G1
• 分類程式碼代碼: 12352204
• NACRES: NA.32

屬性

• 生物源: human
• 化驗: ≥95% (SDS-PAGE)
• 形狀: frozen liquid
• 分子量: 100 kDa
• 包裝: pkg of 1 mg
• UniProt登錄號: P05155
• 運輸包裝: dry ice
• 儲存溫度: −70°C
• 基因資訊: human ... Serpin G1(710)

描述

一般說明

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.

生化／生理作用

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.

外觀

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

安全資訊

• 儲存類別代碼: 11 - Combustible Solids
• 水污染物質分類（WGK）: WGK 3
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable