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Genomes and Genes | J M CooperSummaryAffiliation: University College London Country: UK Publications
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Publications
Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapyJ M Cooper
University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
Eur J Neurol 15:1371-9. 2008..This study investigated the potential for this treatment to modify clinical progression in FRDA in a randomized double blind trial...
Friedreich's ataxia: coenzyme Q10 and vitamin E therapyJ M Cooper
University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, Rowland Hill Street, London NW3 2PF, UK
Mitochondrion 7:S127-35. 2007..The use of quinone therapy has been the most extensively studied to date with clear benefits demonstrated using evaluations of both disease biomarkers and clinical symptoms, and this is the topic that will be covered in this review...- Platelet mitochondrial function in Leber's hereditary optic neuropathyP R Smith
Department of Neurosciences, Royal Free Hospital School of Medicine, London, UK
J Neurol Sci 122:80-3. 1994..002) in those with this mutation who smoked. This reflects an increase in mitochondrial mass with the 11,778 mutation. This effect was not observed with the 3460 mutation even though the complex deficiency was much more severe... - Friedreich's Ataxia: disease mechanisms, antioxidant and Coenzyme Q10 therapyJ M Cooper
University Department of Clinical Neurosciences, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
Biofactors 18:163-71. 2003..Idebenone clearly had an impact upon the cardiac hypertrophy in the majority of patients, although there have not been any other significant benefits reported to date... - The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathyH R Cock
University Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK
Ann Neurol 44:187-93. 1998..These results suggest that the nuclear environment can influence the expression of the biochemical defect in LHON patients with the A3460G mutation... - G209A mutant alpha synuclein expression specifically enhances dopamine induced oxidative damageM Orth
University Department of Clinical Neurosciences, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
Neurochem Int 45:669-76. 2004..This may result in increased dopamine metabolism and cause selective oxidative damage to dopaminergic cells... - Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathyH R Cock
University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
J Neurol Sci 165:10-7. 1999..These findings have important implications for our understanding of complex I dysfunction in the pathogenesis of 3460 Leber's hereditary optic neuropathy... - Role of oxidative damage in Friedreich's ataxiaJ L Bradley
Department of Clinical Neuroscience, Royal Free and University College Medical School, University College London, Rowland Hill Street, London NW3 2PF, United Kingdom
Neurochem Res 29:561-7. 2004..We propose that abnormal respiratory chain function and iron accumulation may lead to a progressive increase in oxidative damage, but increased sensitivity to free radicals may not require detectable respiratory chain dysfunction... - Friedreich's ataxiaJ M Cooper
Department of Clinical Neuroscience, Royal Free and University College Medical School, London, NW3 2PF, United Kingdom
Int Rev Neurobiol 53:147-73. 2002 - Alpha-synuclein expression in HEK293 cells enhances the mitochondrial sensitivity to rotenoneM Orth
University Department of Clinical Neuroscience, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
Neurosci Lett 351:29-32. 2003.... - Molecular mechanisms in mitochondrial DNA depletion syndromeJ W Taanman
Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK
Hum Mol Genet 6:935-42. 1997..Transfer of patient mitochondria with residual mtDNA levels to control cells devoid of mtDNA (rho0 cells) led to restoration of mtDNA levels and, hence, suggests a nuclear involvement in the depletion... - Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesisM A Pook
Division of Biomedical Sciences, Imperial College School of Medicine, London, UK
Neurogenetics 3:185-93. 2001..Our studies are of immediate consequence for the generation of Friedreich's ataxia transgenic mouse models, and further contribute to the accumulating knowledge of human-mouse functional gene replacement systems... - Inclusion formation in Huntington's disease R6/2 mouse muscle culturesM Orth
University Department of Clinical Neurosciences, Royal Free and University College Medical College, University College London, UK
J Neurochem 87:1-6. 2003..Consequently, cultured myotubes from R6/2 mice may be a useful post mitotic cell culture model to study both the biochemical consequences of huntingtin aggregates and the factors that may influence aggregate formation... - Nitric oxide enhances MPP(+) inhibition of complex IM W Cleeter
University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
FEBS Lett 504:50-2. 2001..Thus, NO(*-) may 'prime' the respiratory chain to the effects of MPP(+). These data provide evidence for an interaction between NO(*-) and MPP(+) at the level of the respiratory chain... 
Sensitivity of respiratory chain activities to lipid peroxidation: effect of vitamin E deficiencyR Rafique
University Department of Clinical Neuroscience, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
Biochem J 357:887-92. 2001..This suggests other antioxidants, such as ubiquinol and GSH, may be more important in protecting liver mitochondria and MRC from lipid peroxidation...- Cytochrome oxidase immunohistochemistry: clues for genetic mechanismsS Rahman
Metabolic Unit, Institute of Child Health, University College, London, UK
Brain 123:591-600. 2000..Patients with COX deficiency secondary to mtDNA mutations have a specific pattern of subunit loss, but the majority of children with COX deficiency do not have this pattern of subunit loss and are likely to have nuclear gene defects... - Antibodies to human optic nerve in Leber's hereditary optic neuropathyP R Smith
Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK
J Neurol Sci 130:134-8. 1995..A significant proportion of LHON patients had circulating antibodies to tubulin protein. This finding supports the theory that autoimmunity may play some role in the pathogenesis of LHON... - Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouseS J Tabrizi
University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
Ann Neurol 47:80-6. 2000.... - Mitochondrial dysfunction in Friedreich's ataxia: from pathogenesis to treatment perspectivesR Lodi
Dipartimento di Medicina Clinica e Biotecnologia Applicata, Universita di Bologna, Policlinico S Orsola, Italy
Free Radic Res 36:461-6. 2002..Pilot studies have shown the potential effect of antioxidant therapy based on idebenone or coenzyme Q10 plus Vitamin E administration in this condition and provide a strong rationale for designing larger randomized clinical trials... - Validation of a method for collection and assay of pentane in the exhaled breath of the horseC A Wyse
Department of Veterinary Clinical Studies, Institute of Comparative Medicine, University of Glasgow Veterinary School, Bearsden Road, Bearsden, G61 1QH, Glasgow, UK
Res Vet Sci 76:109-12. 2004.... - Spectroscopic probing of dynamic changes during stimulation and cell remodeling in the single cardiac myocyteO Inya Agha
Department of Electronics, University of Glasgow, Glasgow G12 8LT, Scotland
Anal Chem 79:4581-7. 2007.... - Lab-on-a-chip technologies for proteomic analysis from isolated cellsH Sedgwick
Department of Electronics and Electrical Engineering, University of Glasgow, Glasgow G12 8QQ, UK
J R Soc Interface 5:S123-30. 2008..Using these same trapping electrodes, on-chip electroporation was performed, resulting in cell lysis. Protein release was monitored by confocal fluorescence microscopy...