Open Access. Powered by Scholars. Published by Universities.®
Articles 1 - 1 of 1
Full-Text Articles in Life Sciences
Short-Term Succinic Acid Treatment Mitigates Cerebellar Mitochondrial Oxphos Dysfunction, Neurodegeneration And Ataxia In A Purkinje-Specific Spinocerebellar Ataxia Type 1 (Sca1) Mouse Model, Austin Ferro, Emily Carbone, Jenny Zhang, Evan Marzouk, Monica Villegas, Asher Siegel, Donna Nguyen, Thomas Possidente, Jessilyn Hartman, Kailen Polley, Melissa A. Ingram, Berry Georgia, Thomas H. Reynolds,, Bernard Possidente, Kimberley Frederick, Stephen J. Ives, Sarita Lagalwar
Short-Term Succinic Acid Treatment Mitigates Cerebellar Mitochondrial Oxphos Dysfunction, Neurodegeneration And Ataxia In A Purkinje-Specific Spinocerebellar Ataxia Type 1 (Sca1) Mouse Model, Austin Ferro, Emily Carbone, Jenny Zhang, Evan Marzouk, Monica Villegas, Asher Siegel, Donna Nguyen, Thomas Possidente, Jessilyn Hartman, Kailen Polley, Melissa A. Ingram, Berry Georgia, Thomas H. Reynolds,, Bernard Possidente, Kimberley Frederick, Stephen J. Ives, Sarita Lagalwar
Health and Human Physiological Sciences
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 in cerebellar tissue of a Purkinje cell-driven spinocerebellar ataxia type 1 (SCA1) mouse. Using RNA sequencing transcriptomics, OXPHOS complex assembly analysis and oxygen consumption assays, we report that in the presence of mutant polyglutamine-expanded ataxin-1, SCA1 mice display deficits in cerebellar OXPHOS complex I (NADH-coenzyme Q oxidoreductase). Complex I genes are upregulated at the time of symptom onset and upregulation persists into late …