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Pathology in these animals results from an organ- and cell-specific autoimmune response against normal cardiomyoctes in the atrial and ventricular walls, as well as against very similar myocytes present in the outermost muscle layer surrounding the pulmonary veins. Progression of the autoimmune process could be followed by serial ECG measurements; irradiation of young animals significantly delayed disease progression, and this effect could be reversed by adoptive transfer of splenocytes taken from older animals with complete heart block. Disease progression could also be blocked by cyclosporin A treatment, but was accelerated by injection of complete Fruend's adjuvant. The constellation of findings of spontaneously arising destructive focal lymphocytic infiltrates within the myocardium, rising titers of circulating anticardiac autoantibodies, dilation of the cardiac chambers, and gradual progression to end-stage heart failure bears a striking resemblance to what is seen in humans with idiopathic dilated cardiomyopathy, a serious and often life-threatening medical condition. This transgenic strain provides a highly relevant animal model for human autoimmune myocarditis and postinflammatory dilated cardiomyopathy.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2235552100","type":"journal-article","created":{"date-parts":[[2003,11,16]],"date-time":"2003-11-16T15:42:47Z","timestamp":1068997367000},"page":"13447-13452","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":72,"title":["Autoimmune cardiomyopathy and heart block develop spontaneously in HLA-DQ8 transgenic IA\u03b2 knockout NOD mice"],"prefix":"10.1073","volume":"100","author":[{"given":"John F.","family":"Elliott","sequence":"first","affiliation":[{"name":"Departments of Medical Microbiology and Immunology and Laboratory Medicine and Pathology, Health Sciences Laboratory, Animal Services, Pediatric Cardiology Division of Department of Pediatrics, and Cardiology Division of Department of Medicine, Faculty of Medicine and Dentistry, University 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