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Wiskott‐Aldrich syndrome protein: Emerging mechanisms in immunity
Author(s) -
Rivers Elizabeth,
Thrasher Adrian J.
Publication year - 2017
Publication title -
european journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.272
H-Index - 201
eISSN - 1521-4141
pISSN - 0014-2980
DOI - 10.1002/eji.201646715
Subject(s) - biology , wiskott–aldrich syndrome , immunity , wiskott–aldrich syndrome protein , immunology , immune system , genetics , cytoskeleton , gene , actin cytoskeleton , cell
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.