Mitochondrial ubiquitin ligase MITOL ubiquitinates mutant SOD1 and attenuates mutant SOD1-induced reactive oxygen species generation.

@article{Yonashiro2009MitochondrialUL,
  title={Mitochondrial ubiquitin ligase MITOL ubiquitinates mutant SOD1 and attenuates mutant SOD1-induced reactive oxygen species generation.},
  author={Ryo Yonashiro and Ayumu Sugiura and Misako Miyachi and Toshifumi Fukuda and Nobuko Matsushita and Ryoko Inatome and Yoshinobu Ogata and Takehiro Suzuki and Naoshi Dohmae and Shigeru Yanagi},
  journal={Molecular biology of the cell},
  year={2009},
  volume={20 21},
  pages={
          4524-30
        }
}
We have previously identified a novel mitochondrial ubiquitin ligase, MITOL, which is localized in the mitochondrial outer membrane and is involved in the control of mitochondrial dynamics. In this study, we examined whether MITOL eliminates misfolded proteins localized to mitochondria. Mutant superoxide dismutase1 (mSOD1), one of misfolded proteins, has been shown to localize in mitochondria and induce mitochondrial dysfunction, possibly involving in the onset and progression of amyotrophic… CONTINUE READING
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