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沉默信息调节因子2对SCA3/MJD转基因果蝇的神经保护作用及其与自噬的相关性研究

The Neuro-protective Role of Sir2 in The Process of Neuro-degeneration of The SCA3/MJD Model Flies is Dependent on Autophagy Function

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【作者】曾爱源；朱惊雷；洪亢亢；张灼华；段然慧；孙莉；刘承伟；魏小莉；韦荔莉；陈梅玲；林小慧；陈薇；李清华；

【Author】 ZENG Ai-Yuan1),ZHU Jing-Lei1),HONG Kang-Kang1),ZHANG Zhuo-Hua2),DUAN Ran-Hui2),SUN Li1), LIU Cheng-Wei1),WEI Xiao-Li1),WEI Li-Li1),CHEN Mei-Ling1),LIN Xiao-Hui1),CHEN Wei1),LI Qing-Hua1)(1) Department of Neurology,Affiliated Hospital of Guilin Medical University,Guilin 541001,China; 2) National Laboratory of Medical Genetics of China,Central South University,Changsha 410008,China)

【机构】桂林医学院附属医院神经内科；中南大学医学遗传学国家重点实验室；

【摘要】为探讨沉默信息调节因子2(Sir2)在SCA3/MJD发病机制中的作用.选用GMR-GAL4和Nrv2-GAL4驱动子,利用经典的GAL4-UAS系统,将含有78个CAG重复扩增的ataxin-3蛋白片段(MJDtr-Q78)分别在果蝇眼睛和运动神经元内选择性表达,构建GMR-GAL4/UAS和Nrv2-GAL4/UAS系统SCA3/MJD转基因果蝇模型,然后分别在抑制和不抑制自噬的情况下,使Sir2在SCA3/MJD转基因果蝇眼睛和运动神经元内过表达.结果发现,Sir2过表达明显抑制了SCA3/MJD转基因果蝇眼睛视网膜光感受神经元变性,显著改善了果蝇运动能力,而在自噬被抑制后,Sir2的作用效果明显减弱,表明Sir2对SCA3/MJD转基因果蝇具有神经保护作用,而这种神经保护作用需要依赖自噬的功能.更多还原

【Abstract】 To confer the influence of Sir2 on pathogenesis of SCA3/MJD.GMR-GAL4 and Nrv2-GAL4 system SCA3/MJD transgenic Drosophila models were constructed by using the promoter GMR-GAL4 and Nrv2-GAL4 which drive target selective gene expression in cells of the developing eyes and motor neurons,respectively.Then,Sir2 protein was overexpressed in SCA3/MJD transgenic Drosophila models by genetic methods with or without in a background of RNAi knockdown of Atg7.Overexpression of endogenous Drosophila Sir2 not only notably suppresses the neurotoxicity of MJDtr-Q78 protein,but also significantly improves the movement ability of flies.Moreover,RNAi knockdown of Atg7 significantly Sir2’s protection against SCA3/MJD Drosophila.We confirmed that overexpression of Sir2 could protect SCA3/MJD Drosophila models,and the protection role of Sir2 on SCA3/MJD Drosophila models is autophagy-dependent.更多还原

【关键词】遗传性脊髓小脑性共济失调3型；沉默信息调节因子2(Sir2)；转基因果蝇；自噬；神经保护；
【Key words】 SCA3； ataxin-3； Sir2； transgenic Drosophila models； neuroprotection；

【基金】国家自然科学基金(30960112,81160163);广西自然科学基金(0991259,0899014);广西卫生厅课题(Z2007197);桂林市科技与开发课题(20080707)资助项目~~

【文献出处】生物化学与生物物理进展 ,Progress in Biochemistry and Biophysics , 编辑部邮箱 ,2012年03期

【分类号】R744
【被引频次】1
【下载频次】439

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