Loss of Mitochondrial Ndufs4 in Striatal Medium Spiny Neurons Mediates Progressive Motor Impairment in a Mouse Model of Leigh Syndrome

Inability of mitochondria to generate energy leads to severe and often fatal myoencephalopathies. Among these, Leigh syndrome (LS) is one of the most common childhood mitochondrial diseases; it is characterized by hypotonia, failure to thrive, respiratory insufficiency and progressive mental and mot...

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Detalhes bibliográficos
Publicado no:Front Mol Neurosci
Main Authors: Chen, Byron, Hui, Jessica, Montgomery, Kelsey S., Gella, Alejandro, Bolea, Irene, Sanz, Elisenda, Palmiter, Richard D., Quintana, Albert
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2017
Assuntos:
Neuroscience
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5573716/
https://ncbi.nlm.nih.gov/pubmed/28883788
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fnmol.2017.00265
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