Autoimmune dilated cardiomyopathy in PD-1 receptor-deficient mice
Autoimmune dilated cardiomyopathy in PD-1 receptor-deficient mice
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DOI：
10.1126/science.291.5502.319
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发表时间：
2001-01-12
期刊：
影响因子：
56.9
通讯作者：
Honjo, T
中科院分区：
文献类型：
--
作者：
Nishimura, H;Okazaki, T;Honjo, T
扩张型心肌病是一种严重的心脏病理，病因学知之甚少。在BALB/c小鼠中，但在BALB/c RAG-2（-/-）小鼠中，编码负性免疫调节受体PD-1的基因的破坏引起扩张型心肌病，伴严重收缩受损和充血性心力衰竭猝死。受影响的心脏表现为心肌细胞表面弥漫性免疫球蛋白G（IgG）沉积。所有受影响的PD-1（-/-）小鼠均表现出高滴度的循环IgG自身抗体，该抗体与心肌细胞表面特异性表达的33千道尔顿蛋白质反应。这些结果表明PD-1可能是有助于预防自身免疫性疾病的重要因素。
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.