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Article

Motor neuron disease in transgenic mice with an H46R mutant SOD1 gene.

Journal of neuropathology and experimental neurology - 1 Jun 2007

Sasaki Shoichi, Nagai Makiko, Aoki Masashi, Komori Takashi, Itoyama Yasuto, Iwata Makoto

Abstract excerpt

Human familial amyotrophic lateral sclerosis with an H46R mutant Cu/Zn superoxide dismutase (SOD1) gene is characterized by initial muscle weakness and atrophy in the legs and a very long-term clinical course (approximately 15 years). Transgenic mice with this mutation generated in our laboratory occasionally showed aggregates in the anterior horns and axonal degeneration in all white matter sections of the...

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Source

Identifiers

PMID: 17549011
DOI: 10.1097/01.jnen.0000263868.84188.3b

Genes

SOD1

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