血管性假性血友病因子裂解酶的检测方法及临床应用的研究进展

血管性假性血友病因子裂解酶是存在于正常人血浆中的一种蛋白水解酶,其主要功能是将血浆中的大分子血管性假性血友病因子多聚体裂解成小分子片段,以调节血管性假性血友病因子连接血管下胶原和血小板并诱导血小板黏附和聚集的作用,从而防止微循环内血小板血栓形成。该酶活性的缺陷导致血浆中血管性假性血友病因子质与量发生改变,与血栓性血小板减少性紫癜、肿瘤等疾病的发生、发展有密切关系。因此,血管性假性血友病因子裂解酶的检测及临床应用的研究对于探索血栓性血小板减少性紫癜等疾病的诊断和治疗,具有重要的临床意义。

Detecting Method and Clinical Application of Willebrand Factor Lysase

Von willebrand factor-cleaving protease(vWF-cp) is a proteolytic enzyme residing in normal human plasma.Its main functions are to degrade the large von Willebrand factor(vWF) polymers into smaller fractions,then regulates effect of platelet adhesion and aggregation resulting from conjunction between vWF and collogen under endotheliocyte or platelet,at last,to prevente platelet thrombogenesis in minicirculation..The deficiency of its activity leads to vWF changes in quality and quantity,which are closely related to the genesis and development of thrombotic thrombocytopenic purpura(TTP) and tumors.Therefore,studyies about detection and clinical application of this enzyme have great significance in exploring the diagnosis and treatment of TTP and other diseases.