دورية أكاديمية
Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters
| العنوان: | 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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| المؤلفون: | Cornelius, N, Wardman, JH, Hargreaves, IP, Neergheen, V, Bie, AS, Tümer, Z, Nielsen, JE, Nielsen, TT |
| بيانات النشر: | Elsevier |
| سنة النشر: | 2017 |
| المجموعة: | Liverpool John Moores University: LJMU Research Online |
| مصطلحات موضوعية: | QH426 Genetics |
| الوصف: | 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. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | text |
| اللغة: | English |
| العلاقة: | https://researchonline.ljmu.ac.uk/id/eprint/8734/9/Evidence%20of%20oxidative%20stress%20and%20mitochondrial%20dysfunction%20in%20spinocerebellar%20ataxia%20type%202%20%28SCA2%29%20patient%20fibroblasts%20Effect%20of%20Coenzyme%20Q10%20supplementation%20on%20these%20parameters.pdfTest; 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 |
| DOI: | 10.1016/j.mito.2017.03.001 |
| الإتاحة: | https://doi.org/10.1016/j.mito.2017.03.001Test http://researchonline.ljmu.ac.uk/id/eprint/8734Test/ https://researchonline.ljmu.ac.uk/id/eprint/8734/9/Evidence%20of%20oxidative%20stress%20and%20mitochondrial%20dysfunction%20in%20spinocerebellar%20ataxia%20type%202%20%28SCA2%29%20patient%20fibroblasts%20Effect%20of%20Coenzyme%20Q10%20supplementation%20on%20these%20parameters.pdfTest |
| حقوق: | cc_by_nc_nd |
| رقم الانضمام: | edsbas.DD2D7BAB |
| قاعدة البيانات: | BASE |
| DOI: | 10.1016/j.mito.2017.03.001 |
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