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Wiskott-Aldrich syndrome: a disorder of haematopoietic cytoskeletal regulation.
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- Author(s): Thrasher AJ;Thrasher AJ; Burns S
- Source:
Microscopy research and technique [Microsc Res Tech] 1999 Oct 15; Vol. 47 (2), pp. 107-13.
- Publication Type:
Journal Article; Review
- Language:
English
- Additional Information
- Source:
Publisher: Wiley-Liss Country of Publication: United States NLM ID: 9203012 Publication Model: Print Cited Medium: Print ISSN: 1059-910X (Print) Linking ISSN: 1059910X NLM ISO Abbreviation: Microsc Res Tech Subsets: MEDLINE
- Publication Information:
Original Publication: New York, NY : Wiley-Liss, c1992-
- Subject Terms:
- Abstract:
The Wiskott-Aldrich Syndrome (WAS) is a rare inherited X-linked recessive disease characterised by immune dysregulation and microthrombocytopenia. Recently, the biological mechanisms that are responsible for the pathophysiology of WAS have been shown to be linked to the regulation of the actin cytoskeleton in haematopoietic cells. The WAS protein (WASp) is now known to be a member of a unique family that share similar domain structures, and that are responsible for transduction of signals from the cell membrane to the actin cytoskeleton. The interactions between WASp, the Rho family GTPase Cdc42, and the cytoskeletal organising complex Arp2/3 are probably critical to many of these functions, which, when disturbed, translate into measurable defects of cell polarisation and motility.
(Copyright 1999 Wiley-Liss, Inc.)
- Number of References:
107
- Grant Information:
United Kingdom Wellcome Trust
- Accession Number:
0 (ACTR2 protein, human)
0 (ACTR3 protein, human)
0 (Actin-Related Protein 2)
0 (Actin-Related Protein 3)
0 (Actins)
0 (Carrier Proteins)
0 (Cytoskeletal Proteins)
0 (Fibronectins)
0 (Intracellular Signaling Peptides and Proteins)
0 (Proteins)
0 (WAS protein, human)
0 (WASF1 protein, human)
0 (WIPF1 protein, human)
0 (Wiskott-Aldrich Syndrome Protein)
0 (Wiskott-Aldrich Syndrome Protein Family)
EC 3.6.5.2 (cdc42 GTP-Binding Protein)
- Publication Date:
Date Created: 19991019 Date Completed: 19991209 Latest Revision: 20090929
- Publication Date:
20221213
- Accession Number:
10.1002/(SICI)1097-0029(19991015)47:2<107::AID-JEMT3>3.0.CO;2-H
- Accession Number:
10523789
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