Facial reconstruction

Search LJMU Research Online

Browse Repository | Browse E-Theses

Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters

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

[img]
Preview
Text
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
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: 16 Sep 2018 07:32
DOI or Identification number: 10.1016/j.mito.2017.03.001
URI: http://researchonline.ljmu.ac.uk/id/eprint/8734

Actions (login required)

View Item View Item