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von Willebrand Factor and von Willebrand Disease 被引量:1

von Willebrand Factor and von Willebrand Disease
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摘要 von Willebrand factor (vWF) is an important compound in the human plasma. which is synthesized by endotlrelial cells and also by megakaryocytes. The gene for vWF is located near the tip of the short arm of chromosome 12, being approximately 180 kb in length and consisting of 52 exons separated by 51 introns. The mature vWF subunit consists of 2 050 amino acid residues with molecular weight of about 250 ku. In plasma vWF appears as various multimers. vWF has two well-characterized functions. von Willebrand factor (vWF) is an important compound in the human plasma, which is synthesized by endotlrelial cells and also by megakaryocytes. The gene for vWF is located near the tip of the short arm of chromosome 12, being approximately 180 kb in length and consisting of 52 exons separated by 51 introns. The mature vWF subunit consists of 2 050 amino acid residues with molecular weight of about 250 ku.
作者 王兆钺
出处 《血栓与止血学》 2005年第4期147-149,共3页 Chinese Journal of Thrombosis and Hemostasis
关键词 血液疾病 巨核细胞 染色体 致病因素 出血性疾病 von Willebrand factor von Willebrand factor -cleaving protease von Willebrand disease
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  • 1[1]Wang Y, Li Z, Gu J, et al. von Willebrand disease in China[ J].Chin Med J,2000, 113:677 ~680.
  • 2[2]Mannucci PM. How I treat patients with von Willebrand disease[J].Blood,2001,97:1915 ~ 1919.
  • 3[3]Revel-Vilk S Schmugge M, Carcao MD, et al. Desmopressin (DDAVP) responsiveness in children with von Willebrand diseas [J]. J Pediatr Hematol Oncol, 2003,25:874 ~ 849.

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