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Wiskott-Aldrich syndrome protein: emerging mechanisms in immunity

Rivers, E; Thrasher, AJ; (2017) Wiskott-Aldrich syndrome protein: emerging mechanisms in immunity. European Journal of Immunology , 47 (11) pp. 1857-1866. 10.1002/eji.201646715. Green open access

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Abstract

The Wiskott Aldrich syndrome protein (WASP) participates in innate and adaptive immunity through regulation of actin cytoskeleton-dependent cellular processes, including immune synapse formation, cell signaling, migration and cytokine release. There is also emerging evidence for a direct role in nuclear transcription programmes uncoupled from actin polymerization. A deeper understanding of some of the more complex features of Wiskott Aldrich syndrome (WAS) itself, such as the associated autoimmunity and inflammation, has come from identification of defects in the number and function of anti-inflammatory myeloid cells and regulatory T and B cells, as well as defects in positive and negative B-cell selection. In this review we outline the cellular defects that have been characterized in both human WAS patients and murine models of the disease. We will emphasize in particular recent discoveries that provide a mechanistic insight into disease pathology, including lymphoid and myeloid cell homeostasis, immune synapse assembly and immune cell signaling.

Type: Article
Title: Wiskott-Aldrich syndrome protein: emerging mechanisms in immunity
Location: Germany
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/eji.201646715
Publisher version: http://dx.doi.org/10.1002/eji.201646715
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Autoimmunity; Immune synapse; Inflammation; Wiskott Aldrich syndrome; Wiskott Aldrich syndrome protein
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1571759
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