Actin cytoskeletal defects in immunodeficiency

Dale A. Moulding, Julien Record, Dessislava Malinova, Adrian J. Thrasher*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

73 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)282-299
Number of pages18
JournalImmunological Reviews
Volume256
Issue number1
DOIs
Publication statusPublished - 01 Nov 2013
Externally publishedYes

Keywords

  • Actin cytoskeleton
  • Immunodeficiency
  • WASp neutropenia

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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