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Front Immunol. 2017 May 02;8:490. doi: 10.3389/fimmu.2017.00490. eCollection 2017.

[No title available]

Frontiers in immunology

Maria Carmina Castiello, Francesca Pala, Lucia Sereni, Elena Draghici, Donato Inverso, Aisha V Sauer, Francesca Schena, Elena Fontana, Enrico Radaelli, Paolo Uva, Karla E Cervantes-Luevano, Federica Benvenuti, Pietro L Poliani, Matteo Iannacone, Elisabetta Traggiai, Anna Villa, Marita Bosticardo

Affiliations

  1. San Raffaele Telethon Institute for Gene Therapy (SR-Tiget), Division of Regenerative Medicine, Stem Cells and Gene Therapy, IRCCS San Raffaele Scientific Institute, Milan, Italy.
  2. Vita-Salute San Raffaele University, Milan, Italy.
  3. Dynamics of Immune Responses, Division of Immunology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
  4. Laboratory of Immunology and Rheumatic Disease, IGG, Genova, Italy.
  5. Department of Molecular and Translational Medicine, Pathology Unit, University of Brescia, Brescia, Italy.
  6. VIB11 Center for the Biology of Disease, Center for Human Genetics, KU Leuven, Leuven, Belgium.
  7. CRS4, Science and Technology Park Polaris, Pula, Italy.
  8. Cellular Immunology, International Centre for Genetic Engineering and Biotechnology (ICGEB), Trieste, Italy.
  9. Experimental Imaging Center, IRCCS San Raffaele Scientific Institute, Milan, Italy.
  10. Milan Unit, Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche, Milan, Italy.

PMID: 28512459 PMCID: PMC5411424 DOI: 10.3389/fimmu.2017.00490

Abstract

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency caused by mutations in the gene encoding the hematopoietic-specific WAS protein (WASp). WAS is frequently associated with autoimmunity, indicating a critical role of WASp in maintenance of tolerance. The role of B cells in the induction of autoreactive immune responses in WAS has been investigated in several settings, but the mechanisms leading to the development of autoimmune manifestations have been difficult to evaluate in the mouse models of the disease that do not spontaneously develop autoimmunity. We performed an extensive characterization of

Keywords: B cells; Wiskott–Aldrich syndrome; apoptotic cells; autoimmunity; lymphocytic choriomeningitis virus; toll-like receptors

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