Altered BCR and TLR signals promote enhanced positive selection of autoreactive transitional B cells in Wiskott-Aldrich syndrome

• Published in: The Journal of experimental medicine Vol. 212; no. 10; pp. 1663 - 1677
• Main Authors: Kolhatkar, Nikita S., Brahmandam, Archana, Thouvenel, Christopher D., Becker-Herman, Shirly, Jacobs, Holly M., Schwartz, Marc A., Allenspach, Eric J., Khim, Socheath, Panigrahi, Anil K., Luning Prak, Eline T., Thrasher, Adrian J., Notarangelo, Luigi D., Candotti, Fabio, Torgerson, Troy R., Sanz, Ignacio, Rawlings, David J.
• Format: Journal Article
• Language: English
• Published: United States The Rockefeller University Press 21.09.2015
• Subjects: Adolescent; Adult; Animals; B-Cell Activating Factor - blood; B-Lymphocytes - immunology; B-Lymphocytes - metabolism; B-Lymphocytes - pathology; Case-Control Studies; Child; Child, Preschool; Cytoprotection; High-Throughput Nucleotide Sequencing; Humans; Infant; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Myeloid Differentiation Factor 88 - metabolism; Receptors, Antigen, B-Cell - metabolism; Toll-Like Receptors - metabolism; Wiskott-Aldrich Syndrome - immunology; Wiskott-Aldrich Syndrome - metabolism; Wiskott-Aldrich Syndrome - pathology; Wiskott-Aldrich Syndrome Protein - genetics; Wiskott-Aldrich Syndrome Protein - metabolism; Young Adult
• ISSN: 0022-1007; 1540-9538; 1540-9538
• DOI: 10.1084/jem.20150585

Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency disorder frequently associated with systemic autoimmunity, including autoantibody-mediated cytopenias. WAS protein (WASp)–deficient B cells have increased B cell receptor (BCR) and Toll-like receptor (TLR) signaling, suggesting that thes...