RT Journal Article SR Electronic T1 Autoimmune Dilated Cardiomyopathy in PD-1 Receptor-Deficient Mice JF Science JO Science FD American Association for the Advancement of Science SP 319 OP 322 DO 10.1126/science.291.5502.319 VO 291 IS 5502 A1 Nishimura, Hiroyuki A1 Okazaki, Taku A1 Tanaka, Yoshimasa A1 Nakatani, Kazuki A1 Hara, Masatake A1 Matsumori, Akira A1 Sasayama, Shigetake A1 Mizoguchi, Akira A1 Hiai, Hiroshi A1 Minato, Nagahiro A1 Honjo, Tasuku YR 2001 UL http://science.sciencemag.org/content/291/5502/319.abstract AB Dilated cardiomyopathy is a severe pathology of the heart with poorly understood etiology. Disruption of the gene encoding the negative immunoregulatory receptor PD-1 in BALB/c mice, but not in BALB/c RAG-2−/− mice, caused dilated cardiomyopathy with severely impaired contraction and sudden death by congestive heart failure. Affected hearts showed diffuse deposition of immunoglobulin G (IgG) on the surface of cardiomyocytes. All of the affected PD-1−/− mice exhibited high-titer circulating IgG autoantibodies reactive to a 33-kilodalton protein expressed specifically on the surface of cardiomyocytes. These results indicate that PD-1 may be an important factor contributing to the prevention of autoimmune diseases.