{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:18:27Z","timestamp":1791299907522,"version":"4.1.0"},"reference-count":30,"publisher":"Rockefeller University Press","issue":"3","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2009,3,16]]},"abstract":"<jats:p>Autoimmune-prone nonobese diabetic mice deficient for B7-2 spontaneously develop an autoimmune peripheral neuropathy mediated by inflammatory CD4+ T cells that is reminiscent of Guillain-Barr\u00e9 syndrome and chronic inflammatory demyelinating polyneuropathy. To determine the etiology of this disease, CD4+ T cell hybridomas were generated from inflamed tissue\u2013derived CD4+ T cells. A majority of T cell hybridomas were specific for myelin protein 0 (P0), which was the principal target of autoantibody responses targeting nerve proteins. To determine whether P0-specific T cell responses were sufficient to mediate disease, we generated a novel myelin P0\u2013specific T cell receptor transgenic (POT) mouse. POT T cells were not tolerized or deleted during thymic development and proliferated in response to P0 in vitro. Importantly, when bred onto a recombination activating gene knockout background, POT mice developed a fulminant form of peripheral neuropathy that affected all mice by weaning age and led to their premature death by 3\u20135 wk of age. This abrupt disease was associated with the production of interferon \u03b3 by P0-specific T cells and a lack of CD4+ Foxp3+ regulatory T cells. Collectively, our data suggest that myelin P0 is a major autoantigen in autoimmune peripheral neuropathy.<\/jats:p>","DOI":"10.1084\/jem.20082113","type":"journal-article","created":{"date-parts":[[2009,2,16]],"date-time":"2009-02-16T21:37:43Z","timestamp":1234820263000},"page":"507-514","update-policy":"https:\/\/doi.org\/10.1084\/jem.crossmarkpolicy","source":"Crossref","is-referenced-by-count":49,"title":["A novel myelin P0\u2013specific T cell receptor transgenic mouse develops a fulminant autoimmune peripheral neuropathy"],"prefix":"10.1084","volume":"206","author":[{"given":"C\u00e9dric","family":"Louvet","sequence":"first","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beniwende G.","family":"Kabre","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan W.","family":"Davini","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Martinier","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen A.","family":"Su","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason J.","family":"DeVoss","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy L.","family":"Rosenthal","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark S.","family":"Anderson","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e8ne","family":"Bour-Jordan","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey A.","family":"Bluestone","sequence":"additional","affiliation":[{"name":"Diabetes Center and the Department of Medicine, University of California, San Francisco, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2009,2,16]]},"reference":[{"key":"2023072920074179500_bib1","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1002\/mus.20076","article-title":"Advances in understanding and treatment of immune-mediated disorders of the peripheral nervous system","volume":"30","author":"Kieseier","year":"2004","journal-title":"Muscle Nerve."},{"key":"2023072920074179500_bib2","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1038\/nri2153","article-title":"Antigen-specific tolerance strategies for the prevention and treatment of autoimmune disease","volume":"7","author":"Miller","year":"2007","journal-title":"Nat. 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