蛋白名称
PROC (light chain, Cleaved-Leu179)
免疫原
Synthesized peptide derived from human PROC (light chain, Cleaved-Leu179)
特异性
This antibody detects endogenous levels of Human PROC (light chain, Cleaved-Leu179, protein was cleaved amino acid sequence between 179-180 )
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
其他名称
Vitamin K-dependent protein C (EC 3.4.21.69;Anticoagulant protein C;Autoprothrombin IIA;Blood coagulation factor XIV) [Cleaved into: Vitamin K-dependent protein C light chain; Vitamin K-dependent protein C heavy chain; Activation peptide]
背景
This gene encodes a vitamin K-dependent plasma glycoprotein. The encoded protein is cleaved to its activated form by the thrombin-thrombomodulin complex. This activated form contains a serine protease domain and functions in degradation of the activated forms of coagulation factors V and VIII. Mutations in this gene have been associated with thrombophilia due to protein C deficiency, neonatal purpura fulminans, and recurrent venous thrombosis.[provided by RefSeq, Dec 2009],
功能
catalytic activity:Degradation of blood coagulation factors Va and VIIIa.,disease:Defects in PROC are the cause of protein C deficiency autosomal dominant (ADPROCD) [MIM:176860]. ADPROCD is a cause of hereditary thrombophilia, a hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Individuals with decreased amounts of protein C are classically referred to as having type I protein C deficiency and those with normal amounts of a functionally defective protein as having type II deficiency.,disease:Defects in PROC are the cause of protein C deficiency autosomal recessive (ARPROCD) [MIM:612304]. ARPROCD results in a thrombotic condition that can manifest as a severe neonatal disorder or as a milder disorder with late-onset thrombophilia. The severe form l