脂质沉积性肌病中自噬和泛素相关蛋白表达研究

目的]探讨自噬相关蛋白和泛素相关蛋白的表达对脂质沉积性肌病(lipid storage myopathy,LSM)的影响.方法]选择2010年1月至2015年12月在本院就诊的LSM患者30例作为本次临床研究对象,采用随机数据表法选择其中15例作为观察组,选取同时段于本院进行健康体检且年龄性别相匹配的15例正常体检者作为对照组,采用Western Blot检测上述两组骨骼肌中自噬相关蛋白和泛素相关蛋白的表达情况.结果]观察组骨骼肌中自噬相关蛋白p62、LC3 Ⅰ的表达水平显著高于对照组,而LC3 Ⅱ的表达水平显著低于对照组,且观察组骨骼肌中LC3 Ⅰ/LC3 Ⅱ的比值明显高于对照组;此外,观察组骨骼肌中泛素蛋白连接酶MuRF1、Astrogin-1和锌指蛋白ZNF216的表达水平均明显高于对照组,差异均具有统计学意义(P<0.05).结论]人体自噬功能的抑制以及泛素化相关蛋白表达水平的升高对脂质沉积性肌病的发生发展具有诱导作用.

Study of Autophagy and Expression of Ubiquitin-related Proteins on Lipid-Storage Myopathy

Objective]To investigate the effects of autophagy and expression of ubiquitin-related proteins on lipid storage myopathy (LSM).Methods]Thirty patients with lipid deposition myopathy who were treated in our hospital from January 2010 to December 2015 were selected as the study subject pool, and 15 cases were selected as the observation group by random data table method.In the same period, 15 healthy subjects who matched the observation group patients in age and sex were selected as the control group.Western blot was used to investigate the expression of autophagy and ubiquitin-related proteins in skeletal muscle tissue.Results] The expression of autophagy-related proteins p62 and LC3 Ⅰ (microtubule-associated protein Ⅰ light chain 3) in the skeletal muscle of the observation group was significantly higher than that of the control group, while the expression level of LC3 Ⅱ was significantly lower than that of the control group;the ratio of LC3 Ⅰ to LC3 Ⅱ in the observation group was significantly higher than that of the control group.In addition, the expression of MuRF1, Astrogin-1 and ZNF216 in the skeletal muscle of the observation group was significantly higher than those in the control group (P<0.05), and the difference was statistically significant (P<0.05).Conclusion]The inhibition of autophagy and the increased expression of ubiquitination-related proteins are involved in the development of lipid-induced myopathy.