Wang Suobin, Shen Dingguo
Department of Neuromuscular Disorders, General Hospital of Chinese People's Liberation Army, Beijing 100853, China.
Zhonghua Yi Xue Za Zhi. 2002 Feb 10;82(3):155-7.
To study the association between dystrophin and neuronal nitric oxide synthase in muscles of progressive muscular dystrophy patients and the role of deficiency of nNOS in pathogenesis of muscular dystrophy.
NADPH diaphorase enzyme histochemistry and anti-nNOS, anti-dystrophin, and anti-alpha, beta, gamma, delta-sarcoglycan antibody immunohistochemistry were used to analyze the muscle specimens from progressive muscular dystrophiy patients.
Both nNOS and dystrophin were absent in the sarcolemma region of Duchenne muscular dystrophy (DMD) patients. Dystrophin was reduced, and nNOS was absent or reduced in the sarcolemma region of Becker muscular dystrophy (BMD) patients. Both nNOS and dystrophin were expressed normally in the sarcolemma region of limb girdle muscular dystrophy (LGMD) patients.
Deficiency of nNOS is associated with deficiency of dystrophin in the sarcolemma. Dystrophin may have a novel role in localizing nNOS to sarcolemma and regulating the expression of nNOS. Aberrant regulation of nNOS may contribute to degeneration of muscle fibers in DMD.
研究进行性肌营养不良患者肌肉中肌营养不良蛋白与神经元型一氧化氮合酶之间的关联，以及神经元型一氧化氮合酶缺乏在肌营养不良发病机制中的作用。
采用NADPH黄递酶组织化学以及抗神经元型一氧化氮合酶、抗肌营养不良蛋白和抗α、β、γ、δ - 肌聚糖抗体免疫组织化学方法分析进行性肌营养不良患者的肌肉标本。
杜兴氏肌营养不良（DMD）患者的肌膜区域中肌营养不良蛋白和神经元型一氧化氮合酶均缺失。贝克氏肌营养不良（BMD）患者的肌膜区域中肌营养不良蛋白减少，神经元型一氧化氮合酶缺失或减少。肢带型肌营养不良（LGMD）患者的肌膜区域中肌营养不良蛋白和神经元型一氧化氮合酶均正常表达。
神经元型一氧化氮合酶缺乏与肌膜中肌营养不良蛋白缺乏相关。肌营养不良蛋白可能在将神经元型一氧化氮合酶定位到肌膜以及调节神经元型一氧化氮合酶的表达方面具有新作用。神经元型一氧化氮合酶的异常调节可能导致DMD中肌纤维的退化。
