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Wiskott-Aldrich 综合征家族细胞骨架调节衔接蛋白在免疫调节中的作用。

Contributions of Wiskott-Aldrich syndrome family cytoskeletal regulatory adapters to immune regulation.

机构信息

Department of Medicine, University of Toronto, Mount Sinai Hospital Samuel Lunenfeld Research Institute, Toronto, ON, Canada.

出版信息

Immunol Rev. 2009 Nov;232(1):175-94. doi: 10.1111/j.1600-065X.2009.00846.x.

Abstract

Cytoskeletal structure and dynamic rearrangement are integrally involved in coupling external stimuli to the orchestrated network of molecular interactions and cellular responses required for T-cell effector function. Members of the Wiskott-Aldrich syndrome protein (WASp) family are now widely recognized as cytoskeletal scaffolding adapters that coordinate the transmission of stimulatory signals to downstream induction of actin remodeling and cytoskeletal-dependent T-cell responses. In this review, we discuss the structural and functional properties of the WASp family members, with an emphasis on the roles of these proteins in the molecular pathways underpinning T-cell activation. The contributions of WASp family proteins and the cytoskeletal reorganization they evoke to expression of specific T-cell effector functions and the implications of such activity to normal immune responses and to the immunologic deficits manifested by Wiskott-Aldrich syndrome patients are also described.

摘要

细胞骨架结构和动态重排与协调的分子相互作用网络以及 T 细胞效应功能所需的细胞反应耦联,这其中涉及到细胞骨架。Wiskott-Aldrich 综合征蛋白 (WASp) 家族成员现在被广泛认为是细胞骨架支架接头,它们协调将刺激信号传递到下游诱导肌动蛋白重塑和细胞骨架依赖的 T 细胞反应。在这篇综述中,我们讨论了 WASp 家族成员的结构和功能特性,重点介绍了这些蛋白质在支持 T 细胞激活的分子途径中的作用。还描述了 WASp 家族蛋白的贡献以及它们引发的细胞骨架重排对特定 T 细胞效应功能的表达的影响,以及这种活性对正常免疫反应和 Wiskott-Aldrich 综合征患者表现出的免疫缺陷的影响。

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