Wiskott-Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
Wiskott-Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
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DOI：
10.1016/j.imbio.2009.06.009
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发表时间：
2009-09-01
期刊：
影响因子：
2.8
通讯作者：
Thrasher, Adrian J.
中科院分区：
文献类型：
--
作者：
Bouma, Gerben;Burns, Siobhan O.;Thrasher, Adrian J.
肌动蛋白细胞骨架的调节对于免疫细胞的正确和协作功能的许多方面至关重要，例如迁移、抗原摄取和细胞激活。威斯科特-奥尔德里奇综合征蛋白 (WASp) 是肌动蛋白细胞骨架重排的重要调节因子，缺乏该蛋白会导致免疫功能受损。这篇综述讨论了 WASp 在分子和细胞水平上的作用的最新见解，并评估了 WASp 缺陷如何影响重要的免疫学特征以及免疫细胞功能缺陷如何导致宿主防御受损。 (C) 2009 爱思唯尔有限公司。版权所有。
Regulation of the actin cytoskeleton is crucial for many aspects of correct and cooperative functioning of immune Cells, Such as migration, antigen uptake and cell activation. The Wiskott-Aldrich Syndrome protein (WASp) is an important regulator of actin cytoskeletal rearrangements and lack of this protein results in impaired immune function. This review discusses recent new insights of the role of WASp at molecular and cellular level and evaluates how WASp deficiency affects important immunological features and how defective immune cell function contributes to compromised host defence. (C) 2009 Elsevier GmbH. All rights reserved.