国际神经病学神经外科学杂志2017，Vol.44Issue(4)：365-369,5.DOI:10.16636/j.cnki.jinn.2017.04.005
自噬在Duchenne型肌营养不良中的研究
Role of autophagy in Duchenne muscular dystrophy
摘要
Abstract
Objective To investigate the expression of microtubule-associated protein I light chain 3 (LC3) and p62 in skeletal muscle in patients with Duchenne muscular dystrophy (DMD) and the role of autophagy in necrosis of skeletal muscle cells.Methods A total of 81 patients with pathologically confirmed DMD who visited our hospital from January 2008 to May 2015 were enrolled as DMD group,and 6 patients who were suspected of myopathy and had no marked muscle lesions were enrolled as control group.Myocardial enzyme examination,electromyography,histological and enzymatic staining in skeletal muscle biopsy,and anti-dystrophin-N,-C,-R and antidysferlin immunohistochemical staining were performed for all subjects.The expression of LC3 and p62 in skeletal muscle was measured for 6 DMD patients and the control group.Results All of the 81 DMD patients were male with a mean onset age of 4.60 ± 2.35 years,and the initial symptom was mainly muscle weakness in the lower limbs.Serum creatine kinase reached a peak level at an age of 6 ～8 years and then gradually decreased with increased necrosis of myocytes.Histopathological examination showed typical muscular dystrophy in skeletal muscle in DMD patients.Semi-quantitative Western blot showed a reduction in the expression of LC3-Ⅱ and an increase in the expression of p62 in skeletal muscle in DMD patients.Conclusions Autophagy dysfunction may be involved in the pathophysiological process of skeletal muscle cell necrosis in DMD.关键词
Duchenne型肌营养不良症/肌酸激酶/自噬Key words
Duchenne muscular dystrophy/creatine kinase/autophagy引用本文复制引用
周瑾瑕,李东方,毕方方..自噬在Duchenne型肌营养不良中的研究[J].国际神经病学神经外科学杂志,2017,44(4):365-369,5.基金项目
国家自然科学基金(81401065 （）
81571256) （）
