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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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Опубликовано в: :Front Mol Neurosci
Главные авторы: Chen, Byron, Hui, Jessica, Montgomery, Kelsey S., Gella, Alejandro, Bolea, Irene, Sanz, Elisenda, Palmiter, Richard D., Quintana, Albert
Формат: Artigo
Язык:Inglês
Опубликовано: Frontiers Media S.A. 2017
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Online-ссылка: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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