The role of WASp in T cells and B cells.

Abstract

Wiskott-Aldrich syndrome (WAS) is a form of primary immunodeficiency (PIDs) resulting from mutations of the gene that encodes Wiskott-Aldrich syndrome protein (WASp). WASp is the first identified and most widely studied protein belonging to the actin nucleation-promoting factor family and plays significant role in integrating and transforming signals from critical receptors on the cell surface to actin remodeling. WASp functions in immune defense and homeostasis through the regulation of actin cytoskeleton-dependent cellular processes as well as processes uncoupled with actin polymerization like nuclear transcription programs. In this article, we review the mechanisms of WASp activation through an understanding of its structure. We further discuss the role of WASp in adaptive immunity, paying special attention to some recent findings on the crucial role of WASp in the formation of immunological synapse, the regulation of T follicular helper (Tfh) cells and in the prevention of autoimmunity.

• Authors: Xizi Sun, Yin Wei, Pamela P Lee, Boxu Ren, Chaohong Liu
• Journal: Cellular immunology (Cell Immunol) Vol. 341 Pg. 103919 (07 2019) ISSN: 1090-2163 [Electronic] Netherlands
• PMID: 31047647 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2019 Elsevier Inc. All rights reserved.

Chemical References

• WAS protein, human
• Wiskott-Aldrich Syndrome Protein

Topics

• Actin Cytoskeleton (genetics, immunology)
• Adaptive Immunity
• Animals
• Autoimmunity (genetics)
• B-Lymphocytes (immunology, pathology)
• Disease Models, Animal
• Gene Expression Regulation
• Homeostasis (genetics, immunology)
• Humans
• Immunity, Innate
• Immunological Synapses (genetics)
• Mice
• Signal Transduction
• T-Lymphocytes, Helper-Inducer (immunology, pathology)
• Wiskott-Aldrich Syndrome (genetics, immunology, pathology)
• Wiskott-Aldrich Syndrome Protein (genetics, immunology)