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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Detalles Bibliográficos
Autores principales: Chen, Byron, Hui, Jessica, Montgomery, Kelsey S., Gella, Alejandro, Bolea, Irene, Sanz, Elisenda, Palmiter, Richard D., Quintana, Albert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5573716/
https://www.ncbi.nlm.nih.gov/pubmed/28883788
http://dx.doi.org/10.3389/fnmol.2017.00265

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5573716/
https://www.ncbi.nlm.nih.gov/pubmed/28883788
http://dx.doi.org/10.3389/fnmol.2017.00265

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