英文名称
Thrombomodulin Recombinant Rabbit mAb
英文别名
AHUS6; BDCA-3; BDCA3; CD141; THPH12; THRM; TM; TRBM_HUMAN; THBD; Fetomodulin; TRBM_MOUSE; thrombomodulin
免疫原
A synthesized peptide derived from mouse Thrombomodulin: 300-550
纯化方法
affinity purified by Protein A
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
血栓调节蛋白(thrombomodulin,TM)是一种分布于静脉、动脉和毛细血管内皮细胞表面的质膜蛋白。 一般认为:TM是血管内皮损伤的重要参数,也是凝血酶的受体,已知在人类多种正常组织中表达,亦可表达于许多肿瘤组织,TM可能类似于钙粘蛋白,是具有凝集素样活性的新一类细胞粘附分子的成员。TM是血管内皮细胞膜上的凝血酶受体之一。与凝血酶结合后可降低凝血酶的凝血活性,而加强其激活蛋白C的活性。由于被激活的蛋白C具有抗凝作用,因此,TM是使凝血酶由促凝转向抗凝的重要的血管内凝血抑制因子。
背景资料
Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated.
亚细胞定位
Membrane; Single-pass type I membrane protein.
组织特异性
Endothelial cells are unique in synthesizing thrombomodulin.
翻译后修饰
N-glycosylated.
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
疾病
Defects in THBD are the cause of thrombophilia due to thrombomodulin defect (THPH12) [MIM:614486]. A hemostatic disorder characterized by a tendency to thrombosis.
Defects in THBD are a cause of susceptibility to hemolytic uremic syndrome atypical type 6 (AHUS6) [MIM:612926]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.
相似性
Contains 1 C-type lectin domain.
Contains 6 EGF-like domains.
功能
Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated.