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The Wiskott-Aldrich syndrome |
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| Authors: | |
| Institution: | (1) Division of Immunology, Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 3615 Civic Center Blvd, 19104 Philadelphia, Pennsylvania, USA;(2) Division of Immunology, Childrens Hospital Boston, Department of Pediatrics, Harvard Medical School, 300 Longwood Ave., 02115 Boston, Massachusetts, USA;(3) Division of Infections and Immunolocal Diseases, Department of Pediatrics, University of British Columbia, 317-2194 Health Sciences Mall, BC VT6123 Vacouver, Canada;(4) Department of Molecular and Cell Biology, Harvard University, 7 Divinity Ave., 02138 Cambridge, Massachusetts, USA |
| Abstract: | 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.Received 28 February 2004; received after revision 15 April 2004; accepted 26 April 2004 |
| Keywords: | Wiskott-Aldrich syndrome WASp cytoskeleton actin primary immunodeficiency |
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