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Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

To explore the lethal, ataxic phenotype of complex I deficiency in Ndufs4 knockout (KO) mice, we inactivated Ndufs4 selectively in neurons and glia (NesKO mice). NesKO mice manifested the same symptoms as KO mice including retarded growth, loss of motor ability, breathing abnormalities, and death by...

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Detalhes bibliográficos
Main Authors: Quintana, Albert, Kruse, Shane E., Kapur, Raj P., Sanz, Elisenda, Palmiter, Richard D.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2010
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2890717/
https://ncbi.nlm.nih.gov/pubmed/20534480
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1006214107
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