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

Nanna Cornelius; Jonathan H Wardman; Iain P Hargreaves; Viruna Neergheen; Anne Sigaard Bie; Zeynep Tümer; Jørgen E Nielsen; Troels T Nielsen

doi:10.1016/j.mito.2017.03.001

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

Nanna Cornelius, Jonathan H Wardman, Iain P Hargreaves, Viruna Neergheen, Anne Sigaard Bie, Zeynep Tümer, Jørgen E Nielsen, Troels T Nielsen

19 Citations (Scopus)

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.

Original language	English
Journal	Mitochondrion
Volume	34
Pages (from-to)	103-114
Number of pages	12
ISSN	1567-7249
DOIs	https://doi.org/10.1016/j.mito.2017.03.001
Publication status	Published - 1 May 2017

Keywords

Adolescent
Adult
Aged
Cells, Cultured
Female
Fibroblasts/pathology
Humans
Male
Middle Aged
Mitochondria/pathology
Oxidative Stress
Spinocerebellar Ataxias/pathology
Ubiquinone/analogs & derivatives
Vitamins/metabolism
Young Adult

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10.1016/j.mito.2017.03.001

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Cornelius, N., Wardman, J. H., Hargreaves, I. P., Neergheen, V., Bie, A. S., Tümer, Z., Nielsen, J. E., & Nielsen, T. T. (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, 103-114. https://doi.org/10.1016/j.mito.2017.03.001

Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts : Effect of coenzyme Q10 supplementation on these parameters. / Cornelius, Nanna; Wardman, Jonathan H; Hargreaves, Iain P et al.

In: Mitochondrion, Vol. 34, 01.05.2017, p. 103-114.

Research output: Contribution to journal › Journal article › Research › peer-review

Cornelius, N, Wardman, JH, Hargreaves, IP, Neergheen, V, Bie, AS, Tümer, Z , Nielsen, JE & 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, vol. 34, pp. 103-114. https://doi.org/10.1016/j.mito.2017.03.001

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

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.",

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AU - Wardman, Jonathan H

AU - Hargreaves, Iain P

AU - Neergheen, Viruna

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AB - 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.

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KW - Adult

KW - Aged

KW - Cells, Cultured

KW - Female

KW - Fibroblasts/pathology

KW - Humans

KW - Male

KW - Middle Aged

KW - Mitochondria/pathology

KW - Oxidative Stress

KW - Spinocerebellar Ataxias/pathology

KW - Ubiquinone/analogs & derivatives

KW - Vitamins/metabolism

KW - Young Adult

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DO - 10.1016/j.mito.2017.03.001

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ER -

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

Abstract

Keywords

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