Journal
IMMUNOLOGICAL REVIEWS
Volume 256, Issue 1, Pages 282-299Publisher
WILEY
DOI: 10.1111/imr.12114
Keywords
actin cytoskeleton; immunodeficiency; WASp neutropenia
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Funding
- Wellcome Trust (UK)
- Great Ormond Street Hospital Children's Charity (UK)
- European Union [PERSIST 222878]
- UCL Grand Challenge Studentship scheme
- Great Ormond Street Hospital Childrens Charity [V1223, V1259] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0611-10001] Funding Source: researchfish
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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.
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