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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Publicado en:Front Mol Neurosci
Autores principales: Chen, Byron, Hui, Jessica, Montgomery, Kelsey S., Gella, Alejandro, Bolea, Irene, Sanz, Elisenda, Palmiter, Richard D., Quintana, Albert
Formato: Artigo
Lenguaje:Inglês
Publicado: Frontiers Media S.A. 2017
Materias:
Neuroscience
Acceso en línea: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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