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Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a <jats:italic>Lrba<\/jats:italic> knockout (<jats:italic>Lrba<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup>) mouse model. LRBA\u2010deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific\u2010pathogen\u2010free conditions, after vaccination with T\u2010dependent and T\u2010independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or <jats:italic>Salmonella Typhimurium<\/jats:italic>. Although <jats:italic>Lrba<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice were able to produce normal serum immunoglobulin M (IgM) and IgG and to mount a specific immune response after immunization, they showed elevated serum and secretory basal IgA levels. LRBA was dispensable for B\u2010 and T\u2010cell development, as well as for <jats:italic>in vitro<\/jats:italic> B\u2010cell proliferation, survival, isotype switching and plasmablast differentiation. Interestingly, <jats:italic>Lrba<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice displayed decreased cytotoxic T\u2010lymphocyte\u2010associated protein\u20104 (CTLA\u20104) expression by regulatory T cells and activated conventional CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup> T lymphocytes, reduced frequency of peritoneal B\u20101a cells along with diminished interleukin\u201010 production and increased percentages of T follicular helper cells in Peyer's patches, but without developing overt signs of autoimmunity. Our findings expand the role of LRBA in immune regulatory mechanisms previously reported in patients, and suggest a novel role in IgA production that is crucial for the protection of mucosal surfaces and gut\u2010associated immune tolerance.<\/jats:p>","DOI":"10.1038\/icb.2017.52","type":"journal-article","created":{"date-parts":[[2017,6,27]],"date-time":"2017-06-27T08:21:00Z","timestamp":1498551660000},"page":"789-802","source":"Crossref","is-referenced-by-count":33,"title":["Immunological phenotype of the murine <i>Lrba<\/i> knockout"],"prefix":"10.1111","volume":"95","author":[{"given":"Laura","family":"G\u00e1mez\u2010D\u00edaz","sequence":"first","affiliation":[{"name":"Center for Chronic Immunodeficiency (CCI), Medical Center\u2014Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"Faculty of Biology, University of Freiburg  Freiburg 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