{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T12:22:05Z","timestamp":1781871725558,"version":"3.54.5"},"reference-count":44,"publisher":"Wiley","issue":"23","license":[{"start":{"date-parts":[[2011,10,25]],"date-time":"2011-10-25T00:00:00Z","timestamp":1319500800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FEBS Letters"],"published-print":{"date-parts":[[2011,12]]},"abstract":"<jats:p>Wiskott\u2013Aldrich Syndrome (WAS) is a X\u2010linked primary immunodeficiency disorder also marked by a very high (up to 70%) incidence of autoimmunity. Wiskott\u2013Aldrich Syndrome arises from mutations in the Wiskott\u2013Aldrich Syndrome protein (WASp), a cytoplasmic protein that links signaling by cell surface receptors such as the T\u2010cell receptor and integrins to actin polymerization. WASp promotes the functions of multiple cell types that support immune responses, but also is important for the function of regulatory T cells and in TCR\u2010induced apoptosis, two negative mechanisms of immune regulation that maintain peripheral immune tolerance. Here we review the nature of immune defects and autoimmunity in WAS and WASp deficient mice and discuss how this single gene defect can simultaneously impair immune responses to pathogens and promote autoimmunity. The myriad cellular immune defects found in WAS make this Mendelian syndrome an interesting model for the study of more complex immune diseases that arise from the interplay of environmental and multiple genetic risk factors.<\/jats:p>","DOI":"10.1016\/j.febslet.2011.10.031","type":"journal-article","created":{"date-parts":[[2011,10,27]],"date-time":"2011-10-27T01:55:12Z","timestamp":1319680512000},"page":"3710-3714","source":"Crossref","is-referenced-by-count":38,"title":["Wiskott\u2013Aldrich Syndrome at the nexus of autoimmune and primary immunodeficiency diseases"],"prefix":"10.1002","volume":"585","author":[{"given":"Sophia Y.","family":"Cleland","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard 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