Wiskott-Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation.
Regulation of the actin cytoskeleton is crucial for many aspects of correct and cooperative functioning of immune cells, such as migration, antigen uptake and cell activation. The Wiskott-Aldrich Syndrome protein (WASp) is an important regulator of actin cytoskeletal rearrangements and lack of this protein results in impaired immune function. This review discusses recent new insights of the role of WASp at molecular and cellular level and evaluates how WASp deficiency affects important immunological features and how defective immune cell function contributes to compromised host defence.
Bouma
Gerben
G
Centre for Immunodeficiency, UCL Institute of Child Health, London, UK. g.bouma@ich.ucl.ac.uk
Burns
Siobhan O
SO
Thrasher
Adrian J
AJ
D000199:Actins / Q000378:metabolism; D000818:Animals; D002465:Cell Movement / Q000276:immunology; D003713:Dendritic Cells / Q000276:immunology / Q000378:metabolism; D006098:Granulocytes / Q000276:immunology / Q000378:metabolism; D006801:Humans; D054992:Immunological Synapses; D008213:Lymphocyte Activation / Q000276:immunology; D008214:Lymphocytes / Q000276:immunology / Q000378:metabolism; D008264:Macrophages / Q000276:immunology / Q000378:metabolism; D009000:Monocytes / Q000276:immunology / Q000378:metabolism; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology*; D051316:Wiskott-Aldrich Syndrome Protein / Q000172:deficiency*
D000199:Actins; D051316:Wiskott-Aldrich Syndrome Protein