J M Cooper

Summary

Affiliation: University College London
Country: UK

Publications

  1. doi request reprint Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy
    J M Cooper
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Eur J Neurol 15:1371-9. 2008
  2. ncbi request reprint Friedreich's ataxia: coenzyme Q10 and vitamin E therapy
    J 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
  3. ncbi request reprint Platelet mitochondrial function in Leber's hereditary optic neuropathy
    P R Smith
    Department of Neurosciences, Royal Free Hospital School of Medicine, London, UK
    J Neurol Sci 122:80-3. 1994
  4. ncbi request reprint Friedreich's Ataxia: disease mechanisms, antioxidant and Coenzyme Q10 therapy
    J 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
  5. ncbi request reprint The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathy
    H R Cock
    University Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK
    Ann Neurol 44:187-93. 1998
  6. ncbi request reprint G209A mutant alpha synuclein expression specifically enhances dopamine induced oxidative damage
    M 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
  7. ncbi request reprint Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy
    H R Cock
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
    J Neurol Sci 165:10-7. 1999
  8. ncbi request reprint Role of oxidative damage in Friedreich's ataxia
    J 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
  9. ncbi request reprint Friedreich's ataxia
    J 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
  10. ncbi request reprint Alpha-synuclein expression in HEK293 cells enhances the mitochondrial sensitivity to rotenone
    M 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

Collaborators

Detail Information

Publications23

  1. doi request reprint Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy
    J 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...
  2. ncbi request reprint Friedreich's ataxia: coenzyme Q10 and vitamin E therapy
    J 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...
  3. ncbi request reprint Platelet mitochondrial function in Leber's hereditary optic neuropathy
    P 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...
  4. ncbi request reprint Friedreich's Ataxia: disease mechanisms, antioxidant and Coenzyme Q10 therapy
    J 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...
  5. ncbi request reprint The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathy
    H 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...
  6. ncbi request reprint G209A mutant alpha synuclein expression specifically enhances dopamine induced oxidative damage
    M 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...
  7. ncbi request reprint Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy
    H 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...
  8. ncbi request reprint Role of oxidative damage in Friedreich's ataxia
    J 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...
  9. ncbi request reprint Friedreich's ataxia
    J 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
  10. ncbi request reprint Alpha-synuclein expression in HEK293 cells enhances the mitochondrial sensitivity to rotenone
    M 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
    ....
  11. ncbi request reprint Molecular mechanisms in mitochondrial DNA depletion syndrome
    J 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...
  12. ncbi request reprint Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis
    M 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...
  13. ncbi request reprint Nitric oxide enhances MPP(+) inhibition of complex I
    M 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...
  14. ncbi request reprint Inclusion formation in Huntington's disease R6/2 mouse muscle cultures
    M 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...
  15. pmc Sensitivity of respiratory chain activities to lipid peroxidation: effect of vitamin E deficiency
    R 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...
  16. pmc Influence of microRNA deregulation on chaperone-mediated autophagy and α-synuclein pathology in Parkinson's disease
    L Alvarez-Erviti
    Department of Clinical Neuroscience, Institute of Neurology, University College London, London, UK
    Cell Death Dis 4:e545. 2013
    ....
  17. ncbi request reprint Antibodies to human optic nerve in Leber's hereditary optic neuropathy
    P 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...
  18. ncbi request reprint Cytochrome oxidase immunohistochemistry: clues for genetic mechanisms
    S 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...
  19. ncbi request reprint Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
    S J Tabrizi
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
    Ann Neurol 47:80-6. 2000
    ....
  20. ncbi request reprint Mitochondrial dysfunction in Friedreich's ataxia: from pathogenesis to treatment perspectives
    R 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...
  21. ncbi request reprint Validation of a method for collection and assay of pentane in the exhaled breath of the horse
    C 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
    ....
  22. ncbi request reprint Spectroscopic probing of dynamic changes during stimulation and cell remodeling in the single cardiac myocyte
    O Inya-Agha
    Department of Electronics, University of Glasgow, Glasgow G12 8LT, Scotland
    Anal Chem 79:4581-7. 2007
    ....
  23. pmc Lab-on-a-chip technologies for proteomic analysis from isolated cells
    H 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...