Niebrój-Dobosz I, Lukasiuk M
Department of Neurology, Medical Academy, Warsaw, Poland.
J Neurol. 1995 Jan;242(2):82-6. doi: 10.1007/BF00887821.
The Western blotting technique was used to detect parvalbumin and S-100 protein in muscles from 10 Duchenne muscular dystrophy (DD) patients, 13 patients with other muscle diseases and 5 age-matched healthy subjects. DD muscles were found to contain decreased amounts of parvalbumin and the S-100 protein. The parvalbumin level did not relate to the age of the patients and the stage of the disease. The S-100 protein decreased progressively with the age of the patients. In a very advanced DD case the S-100 protein was present in trace amounts. In other primary myopathies, including Becker dystrophy, and neurogenic muscular atrophy both parvalbumin and S-100 protein levels were similar to that observed in healthy subjects. The decrease in the amount of both calcium binding proteins may contribute to the elevation of free intracellular Ca2+ level in the sarcoplasm of dystrophic muscle and would result in abnormalities in processes regulated by these proteins. The mechanism(s) responsible for the decrease of parvalbumin and S-100 protein in DD muscles are discussed.
采用蛋白质印迹技术检测了10例杜氏肌营养不良症(DD)患者、13例其他肌肉疾病患者和5例年龄匹配的健康受试者肌肉中的小清蛋白和S-100蛋白。发现DD患者的肌肉中小清蛋白和S-100蛋白含量降低。小清蛋白水平与患者年龄和疾病阶段无关。S-100蛋白随患者年龄逐渐降低。在一个非常晚期的DD病例中,S-100蛋白仅微量存在。在其他原发性肌病中,包括贝克尔肌营养不良症和神经源性肌肉萎缩,小清蛋白和S-100蛋白水平与健康受试者相似。这两种钙结合蛋白含量的降低可能导致营养不良性肌肉肌浆中细胞内游离Ca2+水平升高,并导致这些蛋白所调节的过程出现异常。文中讨论了DD患者肌肉中小清蛋白和S-100蛋白减少的机制。