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蛋白C在先天性和获得性血栓形成性疾病中的作用。

The role of protein C in congenital and acquired thrombotic disorders.

作者信息

Housholder G T

机构信息

Department of Pharmacology, University of Texas Health Science Center, Houston.

出版信息

J Oral Maxillofac Surg. 1988 Sep;46(9):781-7. doi: 10.1016/0278-2391(88)90189-9.

DOI:10.1016/0278-2391(88)90189-9
PMID:2842484
Abstract

Control of the coagulation pathway requires mechanisms that limit and localize the clotting process. The vascular endothelium has been shown to play an active role in preventing blood clot formation in vivo. The endothelial cell surface contains a thrombin-binding protein, thrombomodulin, which, when complexed with thrombin, activates vitamin K-dependent protein C, an endogenous anticoagulant. Protein C and its cofactor, protein S, inhibit the clotting process by inactivating factors Va and VIIIa and, additionally, enhance the lysis of fibrin. It has been demonstrated by the detection of heterozygous and homozygous protein C-deficient patients with severe thrombotic complications that protein C is a major regulatory protein of hemostasis and thrombosis. This endogenous anticoagulant pathway is providing a better understanding of both congenital and acquired thrombotic disorders.

摘要

凝血途径的控制需要限制和定位凝血过程的机制。血管内皮在体内防止血栓形成方面发挥着积极作用。内皮细胞表面含有一种凝血酶结合蛋白——血栓调节蛋白,它与凝血酶结合后会激活维生素K依赖的蛋白C,一种内源性抗凝剂。蛋白C及其辅因子蛋白S通过使因子Va和VIIIa失活来抑制凝血过程,此外,还能增强纤维蛋白的溶解。通过检测患有严重血栓并发症的杂合子和纯合子蛋白C缺乏患者,已证明蛋白C是止血和血栓形成的主要调节蛋白。这种内源性抗凝途径有助于更好地理解先天性和获得性血栓性疾病。

相似文献

1
The role of protein C in congenital and acquired thrombotic disorders.蛋白C在先天性和获得性血栓形成性疾病中的作用。
J Oral Maxillofac Surg. 1988 Sep;46(9):781-7. doi: 10.1016/0278-2391(88)90189-9.
2
The regulation of natural anticoagulant pathways.天然抗凝途径的调节
Science. 1987 Mar 13;235(4794):1348-52. doi: 10.1126/science.3029867.
3
Thrombomodulin, an endothelial anticoagulant; its structure, function and expression.血栓调节蛋白,一种内皮抗凝剂;其结构、功能与表达
Jpn Circ J. 1992 Feb;56(2):187-91. doi: 10.1253/jcj.56.187.
4
Protein C.蛋白C
Prog Hemost Thromb. 1984;7:25-54.
5
[Protein C, protein S].[蛋白C,蛋白S]
Ann Biol Clin (Paris). 1987;45(2):184-90.
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[Molecular biology of protein C-thrombomodulin pathway. Structure and function, and basic studies on its clinical application].[蛋白C-血栓调节蛋白途径的分子生物学。结构与功能及其临床应用的基础研究]
Nihon Rinsho. 1993 Jun;51(6):1610-9.
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Interference of blood-coagulation vitamin K-dependent proteins in the activation of human protein C. Involvement of the 4-carboxyglutamic acid domain in two distinct interactions with the thrombin-thrombomodulin complex and with phospholipids.血液凝固维生素K依赖蛋白对人蛋白C激活的干扰。4-羧基谷氨酸结构域在与凝血酶-血栓调节蛋白复合物及磷脂的两种不同相互作用中的参与情况。
Biochem J. 1988 Dec 1;256(2):501-7. doi: 10.1042/bj2560501.
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Blood coagulation: hemostasis and thrombin regulation.血液凝固:止血与凝血酶调节。
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