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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
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