QUEEN'S UNIVERSITY.
Blood. 2014 Jul 17;124(3):313-5. doi: 10.1182/blood-2014-06-578955.
In this issue of Blood, Yee et al1 have demonstrated that expression or infusion of a truncated von Willebrand factor (VWF) fragment containing the factor VIII (FVIII)-binding D′D3 region of VWF is sufficient to stabilize endogenous FVIII levels in VWF-deficient mice. In the absence of the carrier function of VWF, FVIII is susceptible to rapid proteolysis and clearance resulting in markedly reduced plasma levels of FVIII that contribute to a bleeding diathesis.
在本期《Blood》杂志中,Yee 等人 1 证实,表达或输注含有 von Willebrand 因子(VWF)VIII 因子(FVIII)结合区 D′D3 区的截断 VWF 片段足以稳定 VWF 缺乏小鼠体内的内源性 FVIII 水平。在没有 VWF 载体功能的情况下,FVIII 容易被迅速蛋白水解和清除,导致 FVIII 的血浆水平显著降低,从而导致出血倾向。