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.

Keywords

Dilated cardiomyopathyAutoantibodyHeart failureCardiomyopathyAutoimmunityReceptorMedicineAutoimmune diseaseAntibodyInternal medicineEndocrinologyImmunology

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Publication Info

Year
2001
Type
article
Volume
291
Issue
5502
Pages
319-322
Citations
1817
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Hiroyuki Nishimura, Taku Okazaki, Yoshimasa Tanaka et al. (2001). Autoimmune Dilated Cardiomyopathy in PD-1 Receptor-Deficient Mice. Science , 291 (5502) , 319-322. https://doi.org/10.1126/science.291.5502.319

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DOI
10.1126/science.291.5502.319