Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.

@article{Vijayvergiya2005MutantSD,
  title={Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.},
  author={Chetan Vijayvergiya and Myron Flint Beal and Jochen Buck and Giovanni Manfredi},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={2005},
  volume={25 10},
  pages={2463-70}
}
An increasing body of evidence suggests that mitochondrial dysfunction plays an important role in the pathogenesis of familial amyotrophic lateral sclerosis associated with "gain of function" mutations in Cu/Zn superoxide dismutase 1 (SOD1). SOD1 is mostly a cytosolic protein, but a portion of SOD1 is localized in mitochondria of patients with familial amyotrophic lateral sclerosis and transgenic mouse models of the disease. Despite the finding that mutant SOD1 localizes in mitochondria, the… CONTINUE READING

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