A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein.

@article{Sugiura2011AMU,
  title={A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein.},
  author={Ayumu Sugiura and Ryo Yonashiro and Toshifumi Fukuda and Nobuko Matsushita and Shun Nagashima and Ryoko Inatome and Shigeru Yanagi},
  journal={Mitochondrion},
  year={2011},
  volume={11 1},
  pages={139-46}
}
Expansion of a polyglutamine tract in ataxin-3 (polyQ) causes Machado-Joseph disease, a late-onset neurodegenerative disorder characterized by ubiquitin-positive aggregate formation. Several lines of evidence demonstrate that polyQ also accumulates in mitochondria and causes mitochondrial dysfunction. To uncover the mechanism of mitochondrial quality-control via the ubiquitin-proteasome pathway, we investigated whether MITOL, a novel mitochondrial ubiquitin ligase localized in the mitochondrial… CONTINUE READING