The Wiskott-Aldrich syndrome: disordered actin dynamics in haematopoietic cells

被引：40

作者：

Thrasher, AJ

Burns, S

Lorenzi, R

Jones, GE

机构：

[1] UCL, Inst Child Hlth, Mol Immunol Unit, London WC1N 1EH, England

[2] UCL, Inst Child Hlth, Mol Haematol Unit, London, England

[3] Kings Coll London, Randall Ctr, London WC2R 2LS, England

来源：

IMMUNOLOGICAL REVIEWS | 2000年 / 178卷

关键词：

D O I：

10.1034/j.1600-065X.2000.17803.x

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

The Wiskott-Aldrich syndrome protein (WASp) is a member of a unique family whose members share similar domain structures and are responsible for the transduction of signals from the cell membrane to the actin cytoskeleton. For WASp, the interactions with Rho family GTPases and the cytoskeletal organising complex Arp2/3 are critical to these functions, which when disturbed translate into abnormalities of haematopoietic cell signaling, polarisation, migration and phagocytosis. This review discusses the evidence for regulation of highly dynamic cytoskeletal structures by WASp and the consequences of disturbed function on some of these processes.

引用

页码：118 / 128

页数：11

共 100 条

[1] Structure of Cdc42 in complex with the GTPase-binding domain of the 'Wiskott-Aldrich syndrome' protein [J].