Abstract：【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.
朱勇,毕方方,隆芬,江林桂,曹惠,叶飞霆,李艳春. 脂质沉积性肌病中自噬和泛素相关蛋白表达研究[J]. 医学临床研究, 2017, 34(7): 1271-1274,1276.
ZHU Yong, BI Fang-fang, LONG Fen,et al. Study of Autophagy and Expression of Ubiquitin-related Proteins on Lipid-Storage Myopathy. JOURNAL OF CLINICAL RESEARCH, 2017, 34(7): 1271-1274,1276.
[1] 姜涛,熊李,漆学良,等. 以横纹肌溶解为表现的脂质沉积性肌病临床、病理与基因改变特点[J].中风与神经疾病杂志,2016,33(1): 60-63. [2] 周正平,肖欣. 脂质沉积性肌病的电镜超微病理诊断[J].电子显微学报,2016,35(3): 258-260. [3] 陈科,程汉华,周荣家. 自噬与泛素化蛋白降解途径的分子机制及其功能[J].遗传,2012,34(1): 5-18. [4] 李程飞,王永春,石菲,等. 回转模拟失重对人脐静脉内皮细胞LC3、p62、p53和磷酸化p70S6K表达的影响[J].航天医学与医学工程,2016,29(5): 313-317. [5] 罗勇. 细胞自噬在姜黄素增强LoVo细胞对伊立替康化疗敏感性中的作用[J].实用医学杂志,2017,33(6): 871-875. [6] Miglietta D. Naproxcinod shows significant advantages over naproxen in the mdx model of Duchenne Muscular Dystrophy[J].Orphanet J Rare Dis,2015, 10: 101. [7] De Palma C, Perrotta C, Pellegrino P, et al. Skeletal muscle homeostasis in duchenne muscular dystrophy: modulating autophagy as a promising therapeutic strategy[J].Front Aging Neurosci,2014, 6: 188. [8] 韩锋锋,徐卫国,崔志磊,等. AKT/FOXOs/Atrogin-1/MuRF1信号通路在慢性阻塞性肺疾病大鼠骨骼肌萎缩中的作用[J].中国呼吸与危重监护杂志,2013,12(3): 217-222. [9] 张海娜,徐永立,王帅,等. 脑缺血再灌注损伤大鼠患侧骨骼肌早期形态及atrogin-1、MuRF-1 mRNA表达的变化[J].中国康复医学杂志,2014,29(7): 610-614. [10] Mincione G, Di Marcantonio MC, Tarantelli C, et al. Identification of the zinc finger 216 (ZNF216) in human carcinoma cells: a potential regulator of EGFR activity[J].Oncotarget,2016, 7(46): 74947-74965. [11] Hishiya A, Iemura S, Natsume T, et al. A novel ubiquitin-binding protein ZNF216 functioning in muscle atrophy[J].EMBO J,2006, 25(3): 554-564. [12] Engel AG, Siekert RG. Lipid storage myopathy responsive to prednisone[J].Arch Neurol,1972, 27(2): 174-181. [13] Engel A G, Angelini C. Carnitine deficiency of human skeletal muscle with associated lipid storage myopathy: a new syndrome[J].Science,1973, 179(4076): 899-902. [14] Li L. The Molecular Mechanism of Glucagon-Like Peptide-1 Therapy in Alzheimer's Disease, Based on a Mechanistic Target of Rapamycin Pathway[J].CNS Drugs,2017, doi: 10.1007.[15] Tang L, Yang F, Fang Z, et al. Resveratrol Ameliorates Alcoholic Fatty Liver by Inducing Autophagy[J].Am J Chin Med,2016, 44(6): 1207-1220. [16] 殷云杰 杨松 陈燕春,等. 泛素-蛋白酶体系统在CD137信号介导的动脉粥样硬化中的作用[J].中华心血管病杂志,2017, 45(4): 349-352. [17] Fujisawa K, Takami T, Matsuzaki A, et al. Evaluation of the effects of L-carnitine on medaka (Oryzias latipes) fatty liver[J].Sci Rep,2017, 7(1): 2749. [18] Herzog K, van Lenthe H, Wanders R, et al. Identification and diagnostic value of phytanoyl- and pristanoyl-carnitine in plasma from patients with peroxisomal disorders[J].Mol Genet Metab,2017, doi: 10.1016.
2017, Vol. 34 
