The neuro-protective role of Sir2 in the process of neuro-degeneration of the SCA3/MJD model flies is dependent on autophagy function

Ai Yeng Zeng, Jing Lei Zhu, Kang Kang Hong, Zhuohua Zhang, Ran Duan, Li Sun, Cheng Wei Liu, Xiao Li Wei, Li Li Wei, Mei Ling Chen, Xiao Hui Lin, Wei Chen, Qing Hua Li

    Research output: Contribution to journalArticlepeer-review

    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.

    Original languageEnglish
    Pages (from-to)250-256
    Number of pages7
    JournalPROGRESS IN BIOCHEMISTRY AND BIOPHYSICS
    Volume39
    Issue number3
    DOIs
    Publication statusPublished - 2012

    Keywords

    • Ataxin-3
    • Neuroprotection
    • SCA3
    • Sir2
    • Transgenic Drosophila models

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