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Cornelius, N, Wardman, JH, Hargreaves, IP, Neergheen, V, Bie, AS, Tümer, Z, Nielsen, JE and Nielsen, TT (2017) Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters. Mitochondrion, 34. pp. 103-114. ISSN 1567-7249
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Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts Effect of Coenzyme Q10 supplementation on these parameters.pdf - Accepted Version Available under License Creative Commons Attribution Non-commercial No Derivatives. Download (726kB) | Preview |
Abstract
Spinocerebellar ataxia type 2 (SCA2) is a rare neurodegenerative disorder caused by a CAG repeat expansion in the ataxin-2 gene. We show increased oxidative stress, abnormalities in the antioxidant system, changes in complexes involved in oxidative phosphorylation and changes in mitochondrial morphology in SCA2 patient fibroblasts compared to controls, and we show that treatment with CoQ10 can partially reverse these changes. Together, our results suggest that oxidative stress and mitochondrial dysfunction may be contributory factors to the pathophysiology of SCA2 and that therapeutic strategies involving manipulation of the antioxidant system could prove to be of clinical benefit.
| Item Type: | Article |
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| Uncontrolled Keywords: | 0604 Genetics |
| Subjects: | Q Science > QH Natural history > QH426 Genetics |
| Divisions: | Pharmacy & Biomolecular Sciences |
| Publisher: | Elsevier |
| Date Deposited: | 31 May 2018 11:43 |
| Last Modified: | 04 Sep 2021 10:27 |
| DOI or ID number: | 10.1016/j.mito.2017.03.001 |
| URI: | https://researchonline.ljmu.ac.uk/id/eprint/8734 |
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