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Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice model

Dominant mutations in Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS), a fatal disorder characterized by the progressive loss of motor neurons. To investigate the role of antioxidant defenses in ALS we used knockout mice for the glutamate-cysteine ligase...

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Detalhes bibliográficos
Main Authors: Vargas, Marcelo R., Johnson, Delinda A., Johnson, Jeffrey A.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3139005/
https://ncbi.nlm.nih.gov/pubmed/21600285
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2011.04.025
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