Mitochondrial dysfunction and oxidative stress contribute to the pathogenesis of spinocerebellar ataxia type 12 (SCA12).

@article{Wang2011MitochondrialDA,
  title={Mitochondrial dysfunction and oxidative stress contribute to the pathogenesis of spinocerebellar ataxia type 12 (SCA12).},
  author={Yu-Chun Wang and Chi-Mei Lee and Li-Ching Lee and Li-Chu Tung and Hsiu-Mei Hsieh-Li and Guey-Jen Lee-Chen and Ming-Tsan Su},
  journal={The Journal of biological chemistry},
  year={2011},
  volume={286 24},
  pages={21742-54}
}
Spinal cerebellar ataxia type 12 (SCA12) has been attributed to the elevated expression of ppp2r2b. To better elucidate the pathomechanism of the neuronal disorder and to search for a pharmacological treatment, Drosophila models of SCA12 were generated by overexpression of a human ppp2r2b and its Drosophila homolog tws. Ectopic expression of ppp2r2b or tws caused various pathological features, including neurodegeneration, apoptosis, and shortened life span. More detailed analysis revealed that… CONTINUE READING
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