N-WASP is required for B-cell–mediated autoimmunity in Wiskott-Aldrich syndrome

Volpi, S. and Santori, E. and Abernethy, K. and Mizui, M. and Dahlberg, C. I. and Recher, M. and Capuder, K. and Csizmadia, E. and Ryan, D. and Mathew, D. and Tsokos, G. C. and Snapper, S. and Westerberg, L. S. and Thrasher, A. J. and Candotti, F. and Notarangelo, L. D.. (2016) N-WASP is required for B-cell–mediated autoimmunity in Wiskott-Aldrich syndrome. Blood, 127 (2). pp. 216-220.

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Official URL: https://edoc.unibas.ch/62402/

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Mutations of the Wiskott-Aldrich syndrome gene (WAS) are responsible for Wiskott-Aldrich syndrome (WAS), a disease characterized by thrombocytopenia, eczema, immunodeficiency, and autoimmunity. Mice with conditional deficiency of Was in B lymphocytes (B/WcKO) have revealed a critical role for WAS protein (WASP) expression in B lymphocytes in the maintenance of immune homeostasis. Neural WASP (N-WASP) is a broadly expressed homolog of WASP, and regulates B-cell signaling by modulating B-cell receptor (BCR) clustering and internalization. We have generated a double conditional mouse lacking both WASP and N-WASP selectively in B lymphocytes (B/DcKO). Compared with B/WcKO mice, B/DcKO mice showed defective B-lymphocyte proliferation and impaired antibody responses to T-cell-dependent antigens, associated with decreased autoantibody production and lack of autoimmune kidney disease. These results demonstrate that N-WASP expression in B lymphocytes is required for the development of autoimmunity of WAS and may represent a novel therapeutic target in WAS.
Faculties and Departments:03 Faculty of Medicine > Departement Biomedizin > Department of Biomedicine, University Hospital Basel > Immunodeficiency (Recher)
UniBasel Contributors:Recher, Mike
Item Type:Article, refereed
Article Subtype:Research Article
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:07 Jan 2019 18:23
Deposited On:07 Jan 2019 18:23

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