Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters
Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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
APA
Vancouver
Author
Bibtex
}
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