Analysis of autophagic dysfunction in skeletal muscles from Pompe disease mice by using blood ubiquitin-protein conjugates

在本研究中，我们尝试用血泛素蛋白偶联物评价庞贝病小鼠骨骼肌的自噬功能障碍。(1)我们发现Pompe病小鼠血浆中58 kDa泛素蛋白偶联物水平在骨骼肌自噬体病理性积累之前升高。(2)我们发现Pompe病小鼠骨骼肌和血浆中58 kDa蛋白水平升高，这些蛋白定位于Pompe病小鼠骨骼肌纤维中积累的自噬体。(3)这些结果提示，分析血浆中58 kDa泛素蛋白偶联物可能能够检测骨骼肌的自噬功能障碍，并且比骨骼肌免疫组织化学分析更敏感。

In this study, we tried to evaluate the autophagic dysfunction in skeletal muscle from Pompe disease mice by using blood ubiquitin-protein conjugates.(1) We found that the levels of 58 kDa ubiquitin-protein conjugates in plasma from Pompe disease mice were increased prior to pathological accumulation of autophagosomes in skeletal muscles.(2) We found that levels of 58 kDa proteins were increased in skeletal muscles as well as plasma from Pompe disease mice, and these proteins were localized to autophagosomes accumulated in skeletal muscle fibers from Pompe disease mice.(3) These findings suggested that analysis of 58 kDa ubiquitin-protein conjugates in plasma may be able to detect the autophagic dysfunction in skeletal muscles and be more sensitive than immunohistochemical analysis of skeletal muscles.