肌萎缩侧索硬化症患者骨骼肌神经末梢中磷酸化TAR DNA结合蛋白43和泛素的表达

Detection of phosphorylated TDP-43 and ubiquitin in nerve terminals of skeletal muscle in patients with amyotrophic lateral sclerosis

目的:检测肌萎缩侧索硬化症(ALS)患者骨骼肌神经末梢中磷酸化TAR DNA结合蛋白43(p TDP-43)和泛素(Ub)的表达情况,探讨其在ALS发病中的作用机制。方法:收集30例ALS患者及22例对照的骨骼肌标本,采用免疫组化法检测p TDP-43及Ub的表达情况。结果:ALS组、对照组p TDP-43、Ub阳性表达率分别为46. 7%、0. 0%和60. 0%、0. 0%,差异有统计学意义(P <0. 001)。结论:骨骼肌神经末梢是部分ALS患者除神经元及神经胶质细胞以外的p TDP-43病理沉积部位,可能参与了ALS的发病。

Aim: To investigate the role of phosphorylated TAR DNA-binding protein 43( p TDP-43) and ubiquitin( Ub) in pathogenesis and mechanisms of amyotrophic lateral sclerosis( ALS) by detecting p TDP-43 and Ub in the muscle biopsy. Methods: A total of 52 skeletal muscle samples from 30 ALS patients and 22 normal controls were collected. Immunohistochemistry was used to verify the pathological expressions of p TDP-43 and Ub in muscle tissue. Results: The positive expression rates of p TDP-43 and Ub in ALS group and normal control group were 46. 7%,0. 0%,and 60. 0%,0. 0%,respectively( P < 0. 001). Conclusion: Nerve terminals in skeletal muscle are additional sites besides neuron and neurogliocyte of p TDP-43 pathology in some ALS patients,which may play a role in the pathogenesis of ALS.