The Wiskott-Aldrich syndrome

被引:0
|
作者
J. S. Orange
K. D. Stone
S. E. Turvey
K. Krzewski
机构
[1] University of Pennsylvania School of Medicine,Division of Immunology, Children’s Hospital of Philadelphia, Department of Pediatrics
[2] Harvard Medical School,Division of Immunology, Children’s Hospital Boston, Department of Pediatrics
[3] University of British Columbia,Division of Infections and Immunolocal Diseases, Department of Pediatrics
[4] Harvard University,Department of Molecular and Cell Biology
关键词
Wiskott-Aldrich syndrome; WASp; cytoskeleton; actin; primary immunodeficiency;
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学科分类号
摘要
The Wiskott-Aldrich Syndrome (WAS) is an inherited immunodeficiency caused by a variety of mutations in the gene encoding the WAS protein (WASp). WASp is expressed in hematopoetic cells and facilitates the reorganization of the actin cytoskeleton in response to many important cell stimuli. Extensive study of WAS and more recently WASp has given great insight into the relevance of this molecule and related molecules to both basic cell biology and human immune defenses.
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页码:2361 / 2385
页数:24
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