Expression of myoglobin gene in skeletal muscle of patients with neuromuscular diseases
Expression of myoglobin gene in skeletal muscle of patients with neuromuscular diseases
复制标题
神经肌肉疾病患者骨骼肌肌红蛋白基因的表达
作者：
H. Kawai;T. Naruo;K. Yoneda;K. Masuda;T. Mitsui;Y. Nishida
应用放射免疫分析法和北方印迹法检测肌红蛋白（Mb）mRNA水平，研究了Duchenne型肌营养不良症（DMD）、多发性肌炎（PM）和肌萎缩侧索硬化症（ALS）患者骨骼肌Mb基因的表达。肌细胞中肌红蛋白浓度（肌红蛋白/非胶原蛋白）在DMD、PM或ALS患者中降低。然而，虽然每Mb mRNA含量的Mb浓度（Mb/Mb mRNA）在DMD和PM患者中降低，但这些值在ALS患者中是正常的。这些结果表明，肌红蛋白的合成增加，在DMD和PM患者的肌肉，但不足以补偿从受影响的肌肉的肌红蛋白的过度损失，和肌萎缩侧索硬化症患者的肌肉中的合成减少。John Wiley & Sons，Inc.
Expression of the myoglobin (Mb) gene in skeletal muscle was studied in patients with Duchenne muscular dystrophy (DMD), polymyositis (PM), or amyotrophic lateral sclerosis (ALS) by measuring Mb concentration by radioimmunoassay and Mb messenger ribonucleic acid (RNA) (MbmRNA) levels by Northern blot analysis. Mb concentrations in the muscle cells (Mb/noncollagenous protein) were decreased in patients with DMD, PM, or ALS. However, while Mb concentrations per MbmRNA content (Mb/MbmRNA) were decreased in DMD and PM patients, these values were normal in ALS patients. These results suggest that Mb synthesis is increased in muscles of DMD and PM patients, but is not sufficient to compensate for the excessive loss of Mb from the affected muscles, and that the synthesis is decreased in the muscles of ALS patients. © 1994 John Wiley & Sons, Inc.