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IL\u201010\u2010producing regulatory B (Breg) cells are emerging as important mediators of immunosuppressive activity. In experimental, antigen\u2010induced arthritis WASp\u2010deficient (WASp knockout [WAS KO]) mice developed exacerbated disease associated with decreased Breg cells and regulatory T (Treg) cells, but increased Th17 cells in knee\u2010draining LNs. Arthritic WAS KO mice showed increased serum levels of B\u2010cell\u2010activating factor, while their B cells were unresponsive in terms of B\u2010cell\u2010activating factor induced survival and IL\u201010 production. Adoptive transfer of WT Breg cells ameliorated arthritis in WAS KO recipients and restored a normal balance of Treg and Th17 cells. Mice with B\u2010cell\u2010restricted WASp deficiency, however, did not develop exacerbated arthritis, despite exhibiting reduced Breg\u2010 and Treg\u2010cell numbers during active disease, and Th17 cells were not increased over equivalent WT levels. These findings support a contributory role for defective Breg cells in the development of WAS\u2010related autoimmunity, but demonstrate that functional competence in other regulatory populations can be compensatory. A properly regulated cytoskeleton is therefore important for normal Breg\u2010cell activity and complementation of defects in this lineage is likely to have important therapeutic benefits.<\/jats:p>","DOI":"10.1002\/eji.201344245","type":"journal-article","created":{"date-parts":[[2014,6,19]],"date-time":"2014-06-19T15:11:20Z","timestamp":1403190680000},"page":"2692-2702","source":"Crossref","is-referenced-by-count":26,"title":["Exacerbated experimental arthritis in Wiskott\u2013Aldrich syndrome protein deficiency: Modulatory role of regulatory B cells"],"prefix":"10.1002","volume":"44","author":[{"given":"Gerben","family":"Bouma","sequence":"first","affiliation":[{"name":"Molecular Immunology Unit UCL Institute of Child Health  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natalie A.","family":"Carter","sequence":"additional","affiliation":[{"name":"Division of Medicine, UCL Centre for Rheumatology Research  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mike","family":"Recher","sequence":"additional","affiliation":[{"name":"Division of Immunology and The Manton Center for Orphan Disease Research, Children's Hospital, Harvard Medical School  Boston MA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dessislava","family":"Malinova","sequence":"additional","affiliation":[{"name":"Molecular Immunology Unit UCL Institute of Child Health  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marsilio","family":"Adriani","sequence":"additional","affiliation":[{"name":"Molecular Immunology Unit UCL Institute of Child Health  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigi D.","family":"Notarangelo","sequence":"additional","affiliation":[{"name":"Division of Immunology and The Manton Center for Orphan Disease Research, Children's Hospital, Harvard Medical School  Boston MA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siobhan O.","family":"Burns","sequence":"additional","affiliation":[{"name":"Molecular Immunology Unit UCL Institute of Child Health  London UK"},{"name":"Department of Immunology Great Ormond Street Hospital NHS Trust  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Mauri","sequence":"additional","affiliation":[{"name":"Division of Medicine, UCL Centre for Rheumatology Research  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrian J.","family":"Thrasher","sequence":"additional","affiliation":[{"name":"Molecular Immunology Unit UCL Institute of Child Health  London UK"},{"name":"Department of Immunology Great Ormond Street Hospital NHS Trust  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,7,15]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-12-115253"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.imbio.2009.06.009"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.111.5.e622"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)82002-5"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri2724"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2007.02.001"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI29539"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20061338"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20061334"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni833"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.184.6.2271"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20021293"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(02)00274-1"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.178.12.7868"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.0803052"},{"key":"e_1_2_7_17_1","first-page":"3420","article-title":"Regulatory B cells inhibit EAE initiation in mice while other B cells promote disease progression","volume":"118","author":"Matsushita T.","year":"2008","journal-title":"J. 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