Sadler J E, Matsushita T, Dong Z, Tuley E A, Westfield L A
Howard Hughes Medical Institute, Department of Medicine, Jewish Hospital of St. Louis, Washington University School of Medicine, MO 63110, USA.
Thromb Haemost. 1995 Jul;74(1):161-6.
The characterization of mutations in von Willebrand disease provides useful insight into the synthesis, structure, and function of von Willebrand factor. This growing body of information has prompted a reclassification of vWD types that is intended to reflect distinct pathophysiologic mechanisms. Despite this apparent progress, many aspects of vWF biology and pathophysiology remain poorly understood. These include the mechanism by which binding of vWF to platelets is induced at sites of vascular injury, and the factors that influence the likelihood of bleeding symptoms in patients with vWD type 1.
血管性血友病中突变的特征分析为血管性血友病因子的合成、结构及功能提供了有用的见解。这些不断积累的信息促使对血管性血友病类型进行重新分类，旨在反映不同的病理生理机制。尽管有这一明显进展，但血管性血友病因子的生物学和病理生理学的许多方面仍了解甚少。这些方面包括血管损伤部位诱导血管性血友病因子与血小板结合的机制，以及影响1型血管性血友病患者出血症状发生可能性的因素。
