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Fatal breathing dysfunction in a mouse model of Leigh syndrome

Leigh syndrome (LS) is a subacute necrotizing encephalomyelopathy with gliosis in several brain regions that usually results in infantile death. Loss of murine Ndufs4, which encodes NADH dehydrogenase (ubiquinone) iron-sulfur protein 4, results in compromised activity of mitochondrial complex I as w...

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Detalhes bibliográficos
Main Authors: Quintana, Albert, Zanella, Sebastien, Koch, Henner, Kruse, Shane E., Lee, Donghoon, Ramirez, Jan M., Palmiter, Richard D.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2012
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3387817/
https://ncbi.nlm.nih.gov/pubmed/22653057
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI62923
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