Wiskott-Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation

被引：73

作者：

Bouma, Gerben ^{[1
]}

Burns, Siobhan O. ^{[1
,2
]}

Thrasher, Adrian J. ^{[1
,2
]}

机构：

[1] UCL Inst Child Hlth, Ctr Immunodeficiency, London WC1N 1EH, England

[2] Great Ormond St Hosp Sick Children, London WC1N 3JH, England

来源：

IMMUNOBIOLOGY | 2009年 / 214卷 / 9-10期

基金：

英国惠康基金;

关键词：

Wiskott-Aldrich Syndrome; WASp; Dendritic cells; Migration; T lymphocytes; Immunological synapse; Cell activation; SYNDROME PROTEIN-DEFICIENCY; MEDIATED GENE-TRANSFER; REGULATORY T-CELLS; APC CONTACT SITE; IMMUNOLOGICAL SYNAPSE; DENDRITIC CELLS; ACTIN POLYMERIZATION; HEMATOPOIETIC-CELLS; LENTIVIRAL VECTORS; CUTTING EDGE;

D O I：

10.1016/j.imbio.2009.06.009

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页码：778 / 790

页数：13

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