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Article

Mitochondrial alterations in transgenic mice with an H46R mutant Cu/Zn superoxide dismutase gene.

Journal of neuropathology and experimental neurology - 1 Apr 2009

Sasaki Shoichi, Aoki Masashi, Nagai Makiko, Kobayashi Makio, Itoyama Yasuto

Abstract excerpt

We examined morphological alterations in the mitochondria in the spinal cord of H46R mutant Cu/Zn superoxide dismutase transgenic mice; these mice serve as a model for human familial amyotrophic lateral sclerosis. The disease in the mice is characterized by initial muscle weakness and atrophy in the legs, very long clinical courses, and widespread pathological changes of the spinal cord that extend beyond the...

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PMID: 19287315
DOI: 10.1097/NEN.0b013e31819ba185

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