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Fibrillar Inclusions and Motor Neuron Degeneration in Transgenic Mice Expressing Superoxide Dismutase 1 with a Disrupted Copper-Binding Site

Mutations in Cu/Zn superoxide dismutase 1 (SOD1) have been linked to dominantly inherited forms of amyotrophic lateral sclerosis (FALS). To test the hypothesis that the toxicity of mutant SOD1 originates in Cu2+-mediated formation of toxic radicals, we generated transgenic mice that express human SO...

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Autors principals: Jiou Wang, Guilian Xu, Victoria Gonzales, Michael Coonfield, David Fromholt, Neal G. Copeland, Nancy A. Jenkins, David R. Borchelt
Format: Artigo
Idioma:Inglês
Publicat: Elsevier 2002-07-01
Col·lecció:Neurobiology of Disease
Accés en línia:http://www.sciencedirect.com/science/article/pii/S0969996102904985
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