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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Bibliografiske detaljer
Udgivet i:Front Mol Neurosci
Main Authors: Chen, Byron, Hui, Jessica, Montgomery, Kelsey S., Gella, Alejandro, Bolea, Irene, Sanz, Elisenda, Palmiter, Richard D., Quintana, Albert
Format: Artigo
Sprog:Inglês
Udgivet: Frontiers Media S.A. 2017
Fag:
Neuroscience
Online adgang: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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