WeibelPalade小体形成和功能研究进展.pdfVIP

HEREDITAS (Beijing) 2009 9 , 31(9): 882―888 ISSN 0253-9772 综 述 DOI: 10.3724/SP.J.1005.2009.00882 Weibel-Palade 小体形成和功能研究进展 喆 张 , 李巍 , 100101 Weibel-Palade 小体(Weibel-Palade body, WPB)是血管内皮细胞一种特殊的分泌性杆状细胞器, 含有多种 生物活性分子, 受到刺激后可以非常迅速的释放这些内容物, 参与止血、炎症和血管生成等生理功能。血管假 性血友病因子(von Willebrand Factor, vWF)作为该细胞器的主要组成分子, 其多聚体以管状形式在WPB 中规则 排列, 促使WPB 独特的棒状形态的形成。伴随WPB 的形成过程, 其他不同的分子如P-选择素、CD63 、Rab27A 、 Rab3D 等相继被运送到WPB 中发挥其重要的功能。文章就近年来有关WPB 形成的机制和功能等方面的进展 进行了讨论。 Weibel-Palade 小体; 血管假性血友病因子; 溶酶体相关细胞器 Formation and function of Weibel-Palade bodies ZHANG Zhe, LI Wei Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing 100101, China Abstract: Weibel-Palade bodies (WPB) are specialized cigar-shaped secretory organelles in endothelial cells, which contain a variety of biologically active molecules. These contents can be released rapidly by stimulation and involved in hemostasis, inflammation and angiogenesis. The main component of WPB is von Willebrand factor (vWF), whose expres- sion and tubulation are necessary for the formation of the unique rod-like WPBs. Different molecules such as vWF, P-selectin, CD63, Rab27A and Rab3D are recruited into WPB mediated by the AP-1, AP-3 or other transport machinery. The underlying mechanism of the formation of WPB remains further investigation, which will gain insights into its function. The molecular mechanism of WPB formation and its function were discussed in this review. Keywords: Weibel-Palade bodies; von Willebrand factor; lysosome-related organelles (Lysosome-related organelles, , LRO) WPB , WPB (Weibel-Palade body) vWF(von Willebrand factor) (Lysosome-associa