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

Kolhatkar NS1, Brahmandam A2, Thouvenel CD2, Becker-Herman S2, Jacobs HM2, Schwartz MA1, Allenspach EJ1, Khim S2,Panigrahi AK3, Luning Prak ET3, Thrasher AJ4, Notarangelo LD5, Candotti F6, Torgerson TR2, Sanz I7, Rawlings DJ8. 1Department of Immunology and Department of Pediatrics, University of Washington School of Medicine, Seattle, WA 98195. 2Department of Immunology and Department of Pediatrics, University of Washington School of Medicine, Seattle, WA 98195 Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA 98101. 3Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104. 4Molecular Immunology Unit, Section of Molecular and Cellular Immunology, Centre for Immunodeficiency, University College London Institute of Child Health, London WC1N 1EH, England, UK. 5Department of Immunology, Children's Hospital Boston, Boston, MA 09210. 6Genetics and Molecular Biology Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892. 7Lowance Center for Human Immunology and Division of Rheumatology, Department of Medicine, Emory University, Atlanta, GA 30322 Lowance Center for Human Immunology and Division of Rheumatology, Department of Medicine, Emory University, Atlanta, GA 30322. 8Department of Immunology and Department of Pediatrics, University of Washington School of Medicine, Seattle, WA 98195 Department of Immunology and Department of Pediatrics, University of Washington School of Medicine, Seattle, WA 98195 Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA 98101