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Actin cytoskeletal defects in immunodeficiency

Moulding, DA; Record, J; Malinova, D; Thrasher, AJ; (2013) Actin cytoskeletal defects in immunodeficiency. Immunological Reviews , 256 (1) 282 - 299. 10.1111/imr.12114. Green open access

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Abstract

The importance of the cytoskeleton in mounting a successful immune response is evident from the wide range of defects that occur in actin-related primary immunodeficiencies (PIDs). Studies of these PIDs have revealed a pivotal role for the actin cytoskeleton in almost all stages of immune system function, from hematopoiesis and immune cell development, through to recruitment, migration, intercellular and intracellular signaling, and activation of both innate and adaptive immune responses. The major focus of this review is the immune defects that result from mutations in the Wiskott-Aldrich syndrome gene (WAS), which have a broad impact on many different processes and give rise to clinically heterogeneous immunodeficiencies. We also discuss other related genetic defects and the possibility of identifying new genetic causes of cytoskeletal immunodeficiency.

Type: Article
Title: Actin cytoskeletal defects in immunodeficiency
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/imr.12114
Publisher version: http://dx.doi.org/10.1111/imr.12114
Language: English
Additional information: © 2013 The Authors. Immunological Reviews published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution License [http://creativecommons.org/licenses/by/3.0/], which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Keywords: WASp neutropenia, actin cytoskeleton, immunodeficiency
UCL classification: UCL
UCL > Provost and Vice Provost Offices
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 Life Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health 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 > Developmental Biology and Cancer Dept
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/1417386
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