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Immunobiology. 2009;214(9-10):778-90. doi: 10.1016/j.imbio.2009.06.009. Epub 2009 Jul 22.

Wiskott-Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation.

Author information

1
Centre for Immunodeficiency, UCL Institute of Child Health, London, UK. g.bouma@ich.ucl.ac.uk

Abstract

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.

PMID:
19628299
PMCID:
PMC2738782
DOI:
10.1016/j.imbio.2009.06.009
[Indexed for MEDLINE]
Free PMC Article

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