Thrasher, AJ and Burns, S (1999) Wiskott-Aldrich syndrome: a disorder of haematopoietic cytoskeletal regulation. Microsc Res Tech , 47 (2) 107 - 113. 10.1002/(SICI)1097-0029(19991015)47:2<107::AID-JEMT3>3.0.CO;2-H.
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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.
| Type: | Article |
|---|---|
| Title: | Wiskott-Aldrich syndrome: a disorder of haematopoietic cytoskeletal regulation. |
| Location: | UNITED STATES |
| DOI: | 10.1002/(SICI)1097-0029(19991015)47:2<107::AID-JEMT3>3.0.CO;2-H |
| Language: | English |
| Keywords: | Actin-Related Protein 2, Actin-Related Protein 3, Actins, Blood Platelets, Carrier Proteins, Cells, Cultured, Chemotaxis, Cytoskeletal Proteins, Cytoskeleton, Dendritic Cells, Fibronectins, Hematopoietic Stem Cells, Humans, Intracellular Signaling Peptides and Proteins, Macrophages, Protein Structure, Tertiary, Proteins, Pseudopodia, Signal Transduction, Wiskott-Aldrich Syndrome, Wiskott-Aldrich Syndrome Protein, Wiskott-Aldrich Syndrome Protein Family, cdc42 GTP-Binding Protein |
| UCL classification: | UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Child Health > Department of Infection and Immunity > ICH - Molecular Immunology Unit |
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