In Vivo Pathogenic Role of Mutant SOD1 Localized in the Mitochondrial Intermembrane Space

Mutations in Cu,Zn superoxide dismutase (SOD1) are associated with familial amyotrophic lateral sclerosis (ALS). Mutant SOD1 causes a complex array of pathological events, through toxic gain of function mechanisms, leading to selective motor neuron degeneration. Mitochondrial dysfunction is among th...

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Autores principales: Igoudjil, Anissa, Magrané, Jordi, Fischer, Lindsey R., Kim, Hyun Jeong, Hervias, Isabel, Dumont, Magali, Cortez, Czrina, Glass, Jonathan D., Starkov, Anatoly A., Manfredi, Giovanni
Formato: Artigo
Lenguaje:Inglês
Publicado: Society for Neuroscience 2011
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Articles
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3249412/
https://ncbi.nlm.nih.gov/pubmed/22049426
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.1965-11.2011
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