生物来源
human
检测方案
≥95% (SDS-PAGE)
形式
frozen liquid
分子量
100 kDa
包装
pkg of 1 mg
UniProt登记号
运输
dry ice
储存温度
−70°C
基因信息
human ... Serpin G1(710)
一般描述
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.
外形
Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
监管及禁止进口产品
In vivo biosynthesis of endogenous and of human C1 inhibitor in transgenic mice: tissue distribution and colocalization of their expression.
Vinci G
Journal of Immunology, 169(10), 5948-5954 (2002)
Ruby H P Law et al.
Genome biology, 7(5), 216-216 (2006-06-02)
Serpins are a broadly distributed family of protease inhibitors that use a conformational change to inhibit target enzymes. They are central in controlling many important proteolytic cascades, including the mammalian coagulation pathways. Serpins are conformationally labile and many of the
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