Article
Genetically decreased spinal cord copper concentration prolongs life in a transgenic mouse model of amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 8 Sept 2004
Kiaei Mahmoud, Bush Ashley I, Morrison Brett M, Morrison John H, Cherny Robert A, Volitakis Irene, Beal M Flint, Gordon Jon W
Abstract excerpt
Mutations in the Cu/Zn superoxide dismutase (SOD1) gene cause familial amyotrophic lateral sclerosis (FALS) by gain of an aberrant function that is not yet well understood. The role of Cu(2+) in mediating the toxicity of mutant SOD1 has been earnestly contested. We tested the in vivo effects of genetically induced copper deprivation on the ALS phenotype of transgenic mice expressing G86R mutant mouse SOD1, a...
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