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

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Standard

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; Neergheen, Viruna; Bie, Anne Sigaard; Tümer, Zeynep; Nielsen, Jørgen E; Nielsen, Troels T.

I: Mitochondrion, Bind 34, 05.2017, s. 103-114.

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt

Harvard

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, bind 34, s. 103-114. https://doi.org/10.1016/j.mito.2017.03.001

APA

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

Vancouver

Cornelius N, Wardman JH, Hargreaves IP, Neergheen V, Bie AS, Tümer Z o.a. Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters. Mitochondrion. 2017 maj;34:103-114. https://doi.org/10.1016/j.mito.2017.03.001

Author

Cornelius, Nanna ; Wardman, Jonathan H ; Hargreaves, Iain P ; Neergheen, Viruna ; Bie, Anne Sigaard ; Tümer, Zeynep ; Nielsen, Jørgen E ; Nielsen, Troels T. / Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts : Effect of coenzyme Q10 supplementation on these parameters. I: Mitochondrion. 2017 ; Bind 34. s. 103-114.

Bibtex

@article{9ba56410cf9d4f2b855f28711b800e72,
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.",
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",
author = "Nanna Cornelius and Wardman, {Jonathan H} and Hargreaves, {Iain P} and Viruna Neergheen and Bie, {Anne Sigaard} and Zeynep T{\"u}mer and Nielsen, {J{\o}rgen E} and Nielsen, {Troels T}",
note = "Copyright {\textcopyright} 2017 Elsevier B.V. and Mitochondria Research Society. All rights reserved.",
year = "2017",
month = may,
doi = "10.1016/j.mito.2017.03.001",
language = "English",
volume = "34",
pages = "103--114",
journal = "Mitochondrion",
issn = "1567-7249",
publisher = "Elsevier",

}

RIS

TY - JOUR

T1 - Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts

T2 - Effect of coenzyme Q10 supplementation on these parameters

AU - Cornelius, Nanna

AU - Wardman, Jonathan H

AU - Hargreaves, Iain P

AU - Neergheen, Viruna

AU - Bie, Anne Sigaard

AU - Tümer, Zeynep

AU - Nielsen, Jørgen E

AU - Nielsen, Troels T

N1 - Copyright © 2017 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

PY - 2017/5

Y1 - 2017/5

N2 - 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.

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.

KW - Adolescent

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

U2 - 10.1016/j.mito.2017.03.001

DO - 10.1016/j.mito.2017.03.001

M3 - Journal article

C2 - 28263872

VL - 34

SP - 103

EP - 114

JO - Mitochondrion

JF - Mitochondrion

SN - 1567-7249

ER -

ID: 195159936