Autoimmune Dilated Cardiomyopathy in PD-1 Receptor-Deficient Mice
Science2001Vol. 291(5502), pp. 319–322
Citations Over TimeTop 1% of 2001 papers
Hiroyuki Nishimura, Taku Okazaki, Yoshimasa Tanaka, Kazuki Nakatani, Masatake Hara, Akira Matsumori, Shigetake Sasayama, Akira Mizoguchi, Hiroshi Hiai, Nagahiro Minato, Tasuku Honjo
Abstract
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.
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