Both neuromuscular junction (NMJ) dysfunction and altered electrophysiological properties of muscle fibers have been reported in amyotrophic lateral sclerosis (ALS) patients. ALS-related preclinical studies typically use rodent SOD1^G93A overexpression models, but translation to the human disease has been challenged. The present work explored NMJ function and cellular electrophysiological properties of muscles fibers in SOD1^G93A overexpression rats.

中文翻译：

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快缩肌膜电导调节的改变不能解释 SOD1G93A 大鼠的肌肉功能下降

在肌萎缩侧索硬化症 (ALS) 患者中报告了神经肌肉接头 (NMJ) 功能障碍和肌纤维电生理特性的改变。ALS 相关的临床前研究通常使用啮齿动物 SOD1 ^G93A过表达模型，但对人类疾病的转化受到了挑战。目前的工作探讨了 SOD1 ^G93A过表达大鼠肌肉纤维的 NMJ 功能和细胞电生理特性。