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 language | English |
|---|---|
| Pages (from-to) | 250-256 |
| Number of pages | 7 |
| Journal | PROGRESS IN BIOCHEMISTRY AND BIOPHYSICS |
| Volume | 39 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - 2012 |
Keywords
- Ataxin-3
- Neuroprotection
- SCA3
- Sir2
- Transgenic Drosophila models