Linking cellular activation to cytoskeletal reorganization: Wiskott-Aldrich syndrome as a model.
The Wiskott-Aldrich syndrome is an inherited X-linked disorder characterized by immune deficiency, eczema, and thrombocytopenia with small platelets. The mutated protein, Wiskott-Aldrich syndrome protein, is an activator of actin cytoskeletal reorganization in hematopoietic cells. Members of the Wiskott-Aldrich syndrome protein family are being shown to be key integrators of cell signalling and cytoskeletal organization in many eukaryotic cell types. This review focuses on recent discoveries that reveal in increasing detail how Wiskott-Aldrich syndrome protein and its related proteins operate.
Stewart
D M
DM
Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA. dln@helix.nih.gov
Tian
L
L
Nelson
D L
DL
D003599:Cytoskeleton / Q000378:metabolism*; D006801:Humans; D008213:Lymphocyte Activation / Q000276:immunology*; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D014923:Wiskott-Aldrich Syndrome / Q000235:genetics / Q000276:immunology / Q000503:physiopathology*; D051316:Wiskott-Aldrich Syndrome Protein
D011506:Proteins; C495767:WAS protein, human; D051316:Wiskott-Aldrich Syndrome Protein