Search

Your search keyword '"Neergheen, Viruna"' showing total 2 results
Start Over Author "Neergheen, Viruna" Publisher elsevier science
2 results on '"Neergheen, Viruna"'

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

Author

Cornelius N, Wardman JH, Hargreaves IP, Neergheen V, Bie AS, Tümer Z, Nielsen JE, and Nielsen TT

Subjects
Adolescent, Adult, Aged, Cells, Cultured, Female, Humans, Male, Middle Aged, Ubiquinone metabolism, Young Adult, Fibroblasts pathology, Mitochondria pathology, Oxidative Stress, Spinocerebellar Ataxias pathology, Ubiquinone analogs & derivatives, Vitamins metabolism
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., (Copyright © 2017 Elsevier B.V. and Mitochondria Research Society. All rights reserved.)

Published
2017
Full Text
View/download PDF

2. Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders.

Author

Yubero D, Montero R, Martín MA, Montoya J, Ribes A, Grazina M, Trevisson E, Rodriguez-Aguilera JC, Hargreaves IP, Salviati L, Navas P, Artuch R, Jou C, Jimenez-Mallebrera C, Nascimento A, Pérez-Dueñas B, Ortez C, Ramos F, Colomer J, O'Callaghan M, Pineda M, García-Cazorla A, Espinós C, Ruiz A, Macaya A, Marcé-Grau A, Garcia-Villoria J, Arias A, Emperador S, Ruiz-Pesini E, Lopez-Gallardo E, Neergheen V, Simões M, Diogo L, Blázquez A, González-Quintana A, Delmiro A, Domínguez-González C, Arenas J, García-Silva MT, Martín E, Quijada P, Hernández-Laín A, Morán M, Rivas Infante E, Ávila Polo R, Paradas Lópe C, Bautista Lorite J, Martínez Fernández EM, Cortés AB, Sánchez-Cuesta A, Cascajo MV, Alcázar M, and Brea-Calvo G

Subjects
Adolescent, Adult, Child, Child, Preschool, Cohort Studies, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Muscles pathology, Prevalence, Skin pathology, Ubiquinone deficiency, Young Adult, Mitochondrial Diseases pathology, Oxidative Phosphorylation, Ubiquinone analogs & derivatives
Abstract

We evaluated the coenzyme Q₁₀ (CoQ) levels in patients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n=72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts. Our findings suggest that secondary CoQ deficiency is a common finding in OXPHOS and non-OXPHOS disorders. We hypothesize that cases of CoQ deficiency associated with OXPHOS defects could be an adaptive mechanism to maintain a balanced OXPHOS, although the mechanisms explaining these deficiencies and the pathophysiological role of secondary CoQ deficiency deserves further investigation., (Copyright © 2016 Elsevier B.V. and Mitochondria Research Society. All rights reserved.)

Published
2016
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results