缺乏蛋白质c的破坏性影响

M. Kooblall, D. Nash, L. O'Connell, P. Branagan
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引用次数: 0

摘要

蛋白质C是一种在肝脏合成的依赖维生素k的抗凝血蛋白。蛋白C激活后,使凝血因子Va和VIIIa失活,这两种因子是凝血酶生成和因子X激活所必需的。大多数遗传性蛋白C缺乏症患者是杂合的,因为遗传缺陷会降低蛋白C水平或活性,或两者兼而有之(即,遗传是常染色体显性的)。许多获得性疾病也可降低蛋白C水平,包括急性血栓形成、弥散性血管内凝血(DIC)、肝脏疾病、维生素K拮抗剂(VKA)抗凝剂、脑膜炎球菌感染等[1]。下面的例子说明了蛋白质C缺乏可能导致的危及生命的情况。
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The devastating effect of protein c deficiency
Protein C is a vitamin K-dependent anticoagulant protein synthesized in the liver. Upon activation, protein C inactivates coagulation factors Va and VIIIa, which are necessary for thrombin generation and factor X activation. Most patients with inherited protein C deficiency are heterozygous for a genetic defect that reduces protein C levels, activity, or both (ie, transmission is autosomal dominant). A number of acquired conditions also can reduce protein C levels, including acute thrombosis, disseminated intravascular coagulation (DIC), liver disease, vitamin K antagonist (VKA) anticoagulants, meningococcal infection, and others [1]. The following case illustrates a life threatening condition that Protein C deficiency can cause.
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