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Short-term succinic acid treatment mitigates cerebellar mitochondrial OXPHOS dysfunction, neurodegeneration and ataxia in a Purkinje-specific spinocerebellar ataxia type 1 (SCA1) mouse model
Mitochondrial dysfunction plays a significant role in neurodegenerative disease including ataxias and other movement disorders, particularly those marked by progressive degeneration in the cerebellum. In this study, we investigate the role of mitochondrial oxidative phosphorylation (OXPHOS) deficits...
Autores principales: | Ferro, Austin, Carbone, Emily, Zhang, Jenny, Marzouk, Evan, Villegas, Monica, Siegel, Asher, Nguyen, Donna, Possidente, Thomas, Hartman, Jessilyn, Polley, Kailen, Ingram, Melissa A., Berry, Georgia, Reynolds, Thomas H., Possidente, Bernard, Frederick, Kimberley, Ives, Stephen, Lagalwar, Sarita |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5718515/ https://www.ncbi.nlm.nih.gov/pubmed/29211771 http://dx.doi.org/10.1371/journal.pone.0188425 |
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