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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...

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Detalles Bibliográficos
Autores principales: Bouma, Gerben, Burns, Siobhan O., Thrasher, Adrian J.
Formato: Texto
Lenguaje:English
Publicado: Elsevier 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2738782/
https://www.ncbi.nlm.nih.gov/pubmed/19628299
http://dx.doi.org/10.1016/j.imbio.2009.06.009
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author Bouma, Gerben
Burns, Siobhan O.
Thrasher, Adrian J.
author_facet Bouma, Gerben
Burns, Siobhan O.
Thrasher, Adrian J.
author_sort Bouma, Gerben
collection PubMed
description 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.
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spelling pubmed-27387822009-09-17 Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation Bouma, Gerben Burns, Siobhan O. Thrasher, Adrian J. Immunobiology Review 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. Elsevier 2009-09 /pmc/articles/PMC2738782/ /pubmed/19628299 http://dx.doi.org/10.1016/j.imbio.2009.06.009 Text en © 2009 Elsevier GmbH. https://creativecommons.org/licenses/by/3.0/ Open Access under CC BY 3.0 (https://creativecommons.org/licenses/by/3.0/) license
spellingShingle Review
Bouma, Gerben
Burns, Siobhan O.
Thrasher, Adrian J.
Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title_full Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title_fullStr Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title_full_unstemmed Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title_short Wiskott–Aldrich Syndrome: Immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
title_sort wiskott–aldrich syndrome: immunodeficiency resulting from defective cell migration and impaired immunostimulatory activation
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2738782/
https://www.ncbi.nlm.nih.gov/pubmed/19628299
http://dx.doi.org/10.1016/j.imbio.2009.06.009
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