A H Schapira

Summary

Affiliation: University College London
Country: UK

Publications

  1. ncbi Neuroprotection in Parkinson disease: mysteries, myths, and misconceptions
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, and Institute of Neurology, Queen Square, London, England
    JAMA 291:358-64. 2004
  2. ncbi Etiology of Parkinson's disease
    Anthony H V Schapira
    Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, United Kingdom
    Neurology 66:S10-23. 2006
  3. pmc Mitochondrial contribution to Parkinson's disease pathogenesis
    Anthony H V Schapira
    Department of Clinical Neurosciences, UCL Institute of Neurology, Rowland Hill Street, London NW3 2PF, UK
    Parkinsons Dis 2011:159160. 2011
  4. pmc Rasagiline protects against alpha-synuclein induced sensitivity to oxidative stress in dopaminergic cells
    K Y Chau
    Department of Clinical Neurosciences, UCL Institute of Neurology, London, United Kingdom
    Neurochem Int 57:525-9. 2010
  5. doi Recent developments in biomarkers in Parkinson disease
    Anthony H V Schapira
    Department of Clinical Neurosciences, UCL Institute of Neurology, London, UK
    Curr Opin Neurol 26:395-400. 2013
  6. doi Long-term efficacy and safety of safinamide as add-on therapy in early Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, University College London, Institute of Neurology, London, UK
    Eur J Neurol 20:271-80. 2013
  7. doi Success rate, efficacy, and safety/tolerability of overnight switching from immediate- to extended-release pramipexole in advanced Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, University College London Institute of Neurology, London, UK
    Eur J Neurol 20:180-7. 2013
  8. doi Mitochondrial diseases
    Anthony H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Lancet 379:1825-34. 2012
  9. pmc Present and future drug treatment for Parkinson's disease
    A H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London NW3 2PF, UK
    J Neurol Neurosurg Psychiatry 76:1472-8. 2005
  10. doi The clinical relevance of levodopa toxicity in the treatment of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 23:S515-20. 2008

Collaborators

Detail Information

Publications110 found, 100 shown here

  1. ncbi Neuroprotection in Parkinson disease: mysteries, myths, and misconceptions
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, and Institute of Neurology, Queen Square, London, England
    JAMA 291:358-64. 2004
    ..We review clinical trials aimed at detecting neuroprotection in Parkinson disease and address the controversies surrounding the interpretation of these studies...
  2. ncbi Etiology of Parkinson's disease
    Anthony H V Schapira
    Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, United Kingdom
    Neurology 66:S10-23. 2006
    ..Therefore, common themes are beginning to emerge in the etiopathogenesis of PD. This bodes well for research focused on the development of treatments that will modify the course of PD...
  3. pmc Mitochondrial contribution to Parkinson's disease pathogenesis
    Anthony H V Schapira
    Department of Clinical Neurosciences, UCL Institute of Neurology, Rowland Hill Street, London NW3 2PF, UK
    Parkinsons Dis 2011:159160. 2011
    ..The turnover of mitochondria by autophagy (mitophagy) has also become a focus of attention. This review summarises recent discoveries in the contribution of mitochondrial abnormalities to PD etiology and pathogenesis...
  4. pmc Rasagiline protects against alpha-synuclein induced sensitivity to oxidative stress in dopaminergic cells
    K Y Chau
    Department of Clinical Neurosciences, UCL Institute of Neurology, London, United Kingdom
    Neurochem Int 57:525-9. 2010
    ..The data are of relevance to the interpretation of the potential mechanisms of action of rasagiline in explaining the results of disease modification trials in PD...
  5. doi Recent developments in biomarkers in Parkinson disease
    Anthony H V Schapira
    Department of Clinical Neurosciences, UCL Institute of Neurology, London, UK
    Curr Opin Neurol 26:395-400. 2013
    ..The latter will be particularly important for the testing of disease-modifying therapies. This review summarizes recent advances in Parkinson disease biomarker development...
  6. doi Long-term efficacy and safety of safinamide as add-on therapy in early Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, University College London, Institute of Neurology, London, UK
    Eur J Neurol 20:271-80. 2013
    ..Safinamide is an α-aminoamide with both dopaminergic and non-dopaminergic mechanisms of action in Phase III clinical development as a once-daily add-on to dopamine agonist (DA) therapy for early Parkinson's disease (PD)...
  7. doi Success rate, efficacy, and safety/tolerability of overnight switching from immediate- to extended-release pramipexole in advanced Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, University College London Institute of Neurology, London, UK
    Eur J Neurol 20:180-7. 2013
    ..The present study assessed, in advanced PD, the success of an overnight switch from adjunctive IR to ER...
  8. doi Mitochondrial diseases
    Anthony H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Lancet 379:1825-34. 2012
    ..Importantly, the mitochondrion is now a target for therapeutic interventions that encompass small molecules, transcriptional regulation, and genetic manipulation, offering opportunities to treat a diverse range of diseases...
  9. pmc Present and future drug treatment for Parkinson's disease
    A H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London NW3 2PF, UK
    J Neurol Neurosurg Psychiatry 76:1472-8. 2005
    ..This article seeks to set current treatment options in context, review emerging and novel drug treatments for PD, and assess the prospects for disease modification. Surgical therapies are not considered...
  10. doi The clinical relevance of levodopa toxicity in the treatment of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 23:S515-20. 2008
    ..Indeed, there is no evidence to indicate that levodopa is toxic to PD patients, and even some suggestion that it may be protective...
  11. doi Mitochondrial dysfunction in neurodegenerative diseases
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, Rowland Hill Street, London, NW3 2PF, UK
    Neurochem Res 33:2502-9. 2008
    ..g. oxidative stress that may contribute to senescence, and environmental toxins that may cause disease either alone or in combination with a genetic predisposition...
  12. doi Neurobiology and treatment of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, University College London, London NW3 2PF, UK
    Trends Pharmacol Sci 30:41-7. 2009
    ..Particular progress has been made in the field of neuroprotection, where novel therapies and clinical trial designs are being tested. This review will focus particularly upon this area...
  13. doi Drug selection and timing of initiation of treatment in early Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neuroscience, Institute of Neurology, London, United Kingdom
    Ann Neurol 64:S47-55. 2008
    ..The most important limiting factor for the use of levodopa is the emergence of motor complications. These are related to a number of factors including the dose of levodopa and the duration of its use...
  14. doi Molecular and clinical pathways to neuroprotection of dopaminergic drugs in Parkinson disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, NW3 2PF, UK
    Neurology 72:S44-50. 2009
    ..The basis for this may lie in the support of basal ganglia compensatory mechanisms and the restoration of normal dopaminergic transmission...
  15. doi Rasagiline in neurodegeneration
    Anthony H V Schapira
    Institute of Neurology, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
    Exp Neurol 212:255-7. 2008
  16. doi Progress in neuroprotection in Parkinson's disease
    A H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Eur J Neurol 15:5-13. 2008
    ..Perhaps the most important and simplest concept for neuroprotection has been the theory that early dopaminergic support for the degenerating dopaminergic system per se provides significant long-term clinical benefit for PD patients...
  17. doi The scientific and clinical basis for future therapies in Parkinson's disease
    A H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Eur J Neurol 15:v. 2008
  18. doi Future directions in the treatment of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, London, UK
    Mov Disord 22:S385-91. 2007
    ..A range of nondopaminergic drugs including alpha 2-adrenergic antagonists, serotoninergics, and adenosine A2a antagonists are in late-stage development for PD and offer benefit for motor symptoms and motor complications...
  19. ncbi Treatment options in the modern management of Parkinson disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, England
    Arch Neurol 64:1083-8. 2007
  20. ncbi Mitochondrial dysfunction in Parkinson's disease
    A H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, Rowland Hill Street, London NW3 2PF, UK
    Cell Death Differ 14:1261-6. 2007
  21. ncbi Novel pharmacological targets for the treatment of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, Rowland Hill Street, London NW3 2PF, UK
    Nat Rev Drug Discov 5:845-54. 2006
    ....
  22. ncbi Timing of treatment initiation in Parkinson's disease: a need for reappraisal?
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, UK
    Ann Neurol 59:559-62. 2006
  23. ncbi Levodopa in the treatment of Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, UK
    Eur J Neurol 16:982-9. 2009
    ..Maintaining good motor function and quality of life remain the primary goals of therapy and the principle that treatment must be tailored to the individual patient's needs is paramount...
  24. ncbi Etiology and pathogenesis of Parkinson disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Neurol Clin 27:583-603, v. 2009
    ..Specific toxins can cause dopaminergic cell death in man and animals, but they probably have limited relevance to the etiology of PD...
  25. doi Perspectives on recent advances in the understanding and treatment of Parkinson's disease
    A H Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Eur J Neurol 16:1090-9. 2009
    ..The value of existing and novel continuous drug delivery systems in PD is seen as providing simplified regimens, maintenance of motor control, reduction in motor complications and improved patient adherence to drug use...
  26. doi Mitochondrial pathology in Parkinson's disease
    Anthony H V Schapira
    Institute of Neurology, University College London, London, United Kingdom
    Mt Sinai J Med 78:872-81. 2011
    ..However, even these additional pathways overlap with each other and with those of mitochondrial dysfunction and oxidative stress. This review explores these concepts with particular relevance to mitochondrial involvement...
  27. doi Aetiopathogenesis of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, Rowland Hill Street, London NW3 2PF, UK
    J Neurol 258:S307-10. 2011
    ..The importance of identifying the molecular and biochemical events that lead to PD lies in the prospect that novel drug targets will emerge and that new compounds will be developed that slow the progression of the disease...
  28. doi Challenges to the development of disease-modifying therapies in Parkinson's disease
    A H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Eur J Neurol 18:16-21. 2011
    ..Significant developments in this area face several challenges including effective disease-modelling systems and clinical trial designs that can enable a true positive result to be obtained in a relatively short period...
  29. doi Rationale for delayed-start study of pramipexole in Parkinson's disease: the PROUD study
    Anthony H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 25:1627-32. 2010
    ..The results of PROUD will provide insight into the potential for early versus delayed treatment with pramipexole to modify motor outcome at 15 months in recently diagnosed PD patients...
  30. doi Safinamide in the treatment of Parkinson's disease
    Anthony H V Schapira
    University College London, Institute of Neurology, Department of Clinical Neurosciences, Rowland Hill Street, London NW3 2PF, UK
    Expert Opin Pharmacother 11:2261-8. 2010
    ..New drugs offering both dopaminergic and non-dopaminergic actions could offer a significant advantage...
  31. ncbi Molecular and clinical prodrome of Parkinson disease: implications for treatment
    Anthony H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Nat Rev Neurol 6:309-17. 2010
    ..The awareness of the early symptomatic period of PD also raises the possibility of providing treatments that not only improve motor function but might also favorably modify outcome...
  32. doi Complex I: inhibitors, inhibition and neurodegeneration
    A H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, Rowland Hill St, London NW3 2PF, UK
    Exp Neurol 224:331-5. 2010
    ..It is possible that these compounds may contribute to the pathogenesis of neurodegenerative disorders, although further work must be done to confirm their potential participation in pathogenesis...
  33. doi Future strategies for neuroprotection in Parkinson's disease
    Anthony H V Schapira
    Department of Clinical Neurosciences, Institute of Neurology, University College London, Rowland Hill Street, London, NW3 2PF, UK
    Neurodegener Dis 7:210-2. 2010
    ..Trials conducted so far have provided some indication of a positive effect, although the mechanism underlying this is not yet known...
  34. doi Neuroprotection in Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Institute of Neurology, UCL, London, UK
    Parkinsonism Relat Disord 15:S41-3. 2009
    ..Several drugs have attracted attention as potential neuroprotective agents in PD. There are numerous studies demonstrating beneficial effects in the laboratory, but clinical efficacy for neuroprotection remains unproven...
  35. ncbi Mitochondria in the aetiology and pathogenesis of Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences and Institute of Neurology, University College London, London, UK
    Lancet Neurol 7:97-109. 2008
    ....
  36. ncbi 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...
  37. pmc Cytochrome c oxidase deficiency associated with the first stop-codon point mutation in human mtDNA
    M G Hanna
    Neurogenetics Section, University Department of Clinical Neurology, Institute of Neurology, London, WC1N 3BG, United Kingdom
    Am J Hum Genet 63:29-36. 1998
    ..The findings reported here represent only the second case of isolated COX deficiency to be defined at the molecular genetic level and reveal a new mutational mechanism in mitochondrial disease...
  38. ncbi Mitochondrial involvement in Parkinson's disease, Huntington's disease, hereditary spastic paraplegia and Friedreich's ataxia
    A H Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF
    Biochim Biophys Acta 1410:159-70. 1999
    ..Clarifying the role of mitochondria in pathogenesis may provide opportunities for the development of treatments designed to reverse or prevent neurodegeneration...
  39. ncbi Mitochondrial dysfunction in neurodegenerative disorders
    A H Schapira
    University Department of Clinical Neurosciences, Royal Free Hospital School of Medicine and University Department of Clinical Neurology, Institute of Neurology, Rowland Hill Street, London NW3 2PF, UK
    Biochim Biophys Acta 1366:225-33. 1998
    ..In any event, mitochondria present an important target for future strategies for 'neuroprotection' to prevent or retard neurodegeneration...
  40. ncbi Mitochondrial disorders
    A H Schapira
    Royal Free Hospital School of Medicine, London, UK
    Curr Opin Neurol 10:43-7. 1997
    ..A potential role for inborn mitochondrial defects in these disorders has not yet been defined but is currently attracting interest...
  41. ncbi 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...
  42. ncbi 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...
  43. ncbi HLA class II genotypes in Leber's hereditary optic neuropathy
    G G Govan
    University Department of Clinical Neurology, Institute of Neurology, London, UK
    J Neurol Sci 126:193-6. 1994
    ..Furthermore, affected relative pairs did not share HLA genotypes more than discordant pairs. We conclude that the HLA-DR locus is not a major genetic determinant for the development of blindness in LHON...
  44. ncbi 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...
  45. ncbi 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...
  46. ncbi Updated guidelines for the management of Parkinson's disease
    K Bhatia
    University Department of Clinical Neurology, Institute of Neurology, London
    Hosp Med 62:456-70. 2001
    ..This article reviews new data and addresses issues left unanswered in the previous guidelines...
  47. ncbi Mitochondria and degenerative disorders
    M Orth
    Department of Clinical Neurosciences, Royal Free and University College London Medical School, UK
    Am J Med Genet 106:27-36. 2001
    ....
  48. ncbi 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...
  49. ncbi Genetic and environmental factors in the cause of Parkinson's disease
    Thomas T Warner
    Department of Clinical Neurosciences, Royal Free and University College Medical School, London NW3 2PF, United Kingdom
    Ann Neurol 53:S16-23; discussion S23-5. 2003
    ..This article reviews the evidence in support of genetic and environmental factors in the cause of PD...
  50. pmc Phenotype, genotype, and worldwide genetic penetrance of LRRK2-associated Parkinson's disease: a case-control study
    Daniel G Healy
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Lancet Neurol 7:583-90. 2008
    ..LRRK2-associated PD be distinguished from idiopathic PD; which mutations in LRRK2 are pathogenic; and what is the age-specific cumulative risk of PD for individuals who inherit or are at risk of inheriting a deleterious mutation in LRRK2?..
  51. pmc Silencing of PINK1 expression affects mitochondrial DNA and oxidative phosphorylation in dopaminergic cells
    Matthew E Gegg
    Department of Clinical Neurosciences, Institute of Neurology, University College London, Queen Square, London, United Kingdom
    PLoS ONE 4:e4756. 2009
    ..The PINK1 protein is a serine/threonine kinase localized in mitochondria and the cytosol. Its precise function is unknown, but it is involved in neuroprotection against a variety of stress signalling pathways...
  52. ncbi Clinical, biochemical and molecular genetic features of Leber's hereditary optic neuropathy
    R M Chalmers
    University Department of Clinical Neurosciences, Royal Free Hospital and University College Medical School of University College London, London NW3 2PF, UK
    Biochim Biophys Acta 1410:147-58. 1999
    ..However, the clinical and experimental data reviewed suggest differences in the phenotype associated with each of the three mutations which may reflect variation in the disease mechanisms resulting in this common end-point...
  53. ncbi Polyphenotypic expression of mitochondrial toxicity caused by nucleoside reverse transcriptase inhibitors
    Robert F Miller
    Department of Sexually Transmitted Diseases, Royal Free and University College Medical School, University College London and Camden Primary Care Trust, London, UK
    Antivir Ther 8:253-7. 2003
    ..At this stage, dual protease inhibitors and nevirapine were started. A repeat muscle biopsy 14 months after presentation showed normal morphology and respiratory chain biochemistry was almost normal...
  54. ncbi Relapsing neuropathy in an 18-year-old woman
    Lionel Ginsberg
    University Department of Clinical Neurosciences, Hampstead Campus, Royal Free and University College Medical School, University College London, UK
    Lancet Neurol 6:192-8. 2007
  55. ncbi Analysis of the trinucleotide CAG repeat from the DNA polymerase gamma gene (POLG) in patients with Parkinson's disease
    Jan Willem Taanman
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
    Neurosci Lett 376:56-9. 2005
    ..Our results rule out POLG CAG repeat instability as a common pathogenic mechanism in idiopathic Parkinson's disease...
  56. ncbi Rationale for the use of dopamine agonists as neuroprotective agents in Parkinson's disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, UCL, Queen Square, London, United Kingdom
    Ann Neurol 53:S149-57; discussion S157-9. 2003
  57. ncbi Mitochondrial dysfunction associated with neuronal death following status epilepticus in rat
    Hannah R Cock
    Department of Clinical and Experimental Epilepsy, Institute of Neurology, University College London, Queen Square, WC1N 3BG, London, UK
    Epilepsy Res 48:157-68. 2002
    ..The pattern of abnormalities is consistent with reversible mechanisms being involved in excitotoxic cell damage. This, together with the timing of changes, suggests new avenues for therapeutic intervention...
  58. doi Characterization of a novel TYMP splice site mutation associated with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)
    Jan Willem Taanman
    Department of Clinical Neurosciences, Institute of Neurology, University College London, Rowland Hill Street, London, United Kingdom
    Neuromuscul Disord 19:151-4. 2009
    ..The patient's fibroblasts showed gradual loss of the mitochondrial DNA-encoded subunit I of cytochrome-c oxidase, suggesting a progressive mitochondrial DNA defect in culture...
  59. ncbi Neuroprotection and dopamine agonists
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, and the Institute of Neurology, University College London, London, UK
    Neurology 58:S9-18. 2002
    ..If the neuroprotective action of this drug is confirmed in patients with PD, this will have important implications for its early use in patients...
  60. ncbi Restless legs syndrome: an update on treatment options
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
    Drugs 64:149-58. 2004
    ..Hypnosedatives also have a role in RLS management. Patients with intractable RLS may require combination treatment. Several systemic disorders can cause RLS, and these should be identified and treated appropriately...
  61. pmc Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy
    Matthew E Gegg
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Hum Mol Genet 19:4861-70. 2010
    ..PINK1 and parkin are thus required for the removal of damaged mitochondria in dopaminergic cells, and inhibition of this pathway may lead to the accumulation of defective mitochondria which may contribute to PD pathogenesis...
  62. doi Analysis of mutant DNA polymerase gamma in patients with mitochondrial DNA depletion
    Jan Willem Taanman
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, United Kingdom
    Hum Mutat 30:248-54. 2009
    ..The assays may facilitate the identification of those patients in whom screening for POLG mutations would be most appropriate...
  63. ncbi Disease modification in Parkinson's disease
    Anthony H V Schapira
    Royal Free and University College Medical School and the Institute of Neurology, University College London, UK
    Lancet Neurol 3:362-8. 2004
    ..The most important challenge, particularly for those drugs that might have a symptomatic effect, is defining appropriate markers that will confirm a neuroprotective effect...
  64. ncbi Mitochondrial disease
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, and Institute of Neurology, University College London, London NW3 2PF, UK
    Lancet 368:70-82. 2006
    ..Improved understanding of mtDNA inheritance and mutation penetrance patterns, and novel techniques for mtDNA modification offer significant prospects for more accurate genetic counselling and effective future therapies...
  65. ncbi Proteasomal inhibition causes loss of nigral tyrosine hydroxylase neurons
    Anthony H V Schapira
    University Department of Clinical Neurosciences, Royal Free and University College, Medical School, London, United Kingdom
    Ann Neurol 60:253-5. 2006
    ..Systemic inhibition of the UPS warrants further evaluation as a means to model Parkinson's disease...
  66. ncbi PINK1-parkin-dependent mitophagy involves ubiquitination of mitofusins 1 and 2: Implications for Parkinson disease pathogenesis
    Matthew E Gegg
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Autophagy 7:243-5. 2011
    ..Here, we discuss how ubiquitination of the mitofusins might facilitate mitochondria degradation and the potential for activating mitophagy as a treatment for diseases affecting brain and muscle...
  67. ncbi Causes of neuronal death in Parkinson's disease
    A H Schapira
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, London, NW3 2PF
    Adv Neurol 86:155-62. 2001
    ..Future research directed toward developing neuroprotective strategies may reduce oxidative stress and stabilize or improve mitochondrial function...
  68. ncbi 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...
  69. ncbi HLA class I genotypes in Leber's hereditary optic neuropathy
    R M Chalmers
    University Department of Clinical Neurology, Institute of Neurology, London, UK
    J Neurol Sci 135:173-5. 1996
    ..There was no association between LHON and any genotype. We conclude that the classical class I MHC loci are not major determinants of the development of blindness in LHON...
  70. ncbi Relationship between alpha synuclein phosphorylation, proteasomal inhibition and cell death: relevance to Parkinson's disease pathogenesis
    Kai Yin Chau
    University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    J Neurochem 110:1005-13. 2009
    ....
  71. doi Non-motor symptoms of Parkinson's disease: dopaminergic pathophysiology and treatment
    K Ray Chaudhuri
    National Parkinson Foundation Centre of Excellence, King s College Hospital and University Hospital Lewisham, London, UK
    Lancet Neurol 8:464-74. 2009
    ..This Review provides a comprehensive overview of the management of this challenging aspect of PD...
  72. ncbi Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up
    Paul E Hart
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, England
    Arch Neurol 62:621-6. 2005
    ....
  73. ncbi Metabolic enzyme expression in dopaminergic neurons in Parkinson's disease: an in situ hybridization study
    A E Kingsbury
    Parkinson's Disease Society Brain Research Centre and Institute of Neurology, University College London, UK
    Ann Neurol 50:142-9. 2001
    ..mitochondrial complex I activity is significantly reduced in IPD, intrinsically low expression of glycolytic enzymes, together with disease-related reduction in complex I activity, may be a contributory factor predisposing nigral neurons..
  74. pmc Salbutamol treatment in a patient with hyperkalaemic periodic paralysis due to a mutation in the skeletal muscle sodium channel gene (SCN4A)
    M G Hanna
    Department of Clinical Neurology, Institute of Neurology, London, UK
    J Neurol Neurosurg Psychiatry 65:248-50. 1998
    ..Treatment with beta-adrenergic agents should be considered in patients with hyperkalaemic periodic paralysis who are intolerant of, or resistant to, diuretic medications...
  75. pmc Neuromyelitis optica (Devic's syndrome): no association with the primary mitochondrial DNA mutations found in Leber hereditary optic neuropathy
    H Cock
    Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK
    J Neurol Neurosurg Psychiatry 62:85-7. 1997
    ..It is concluded that the primary mtDNA mutations currently associated with LHON are not responsible for the prominence of optic nerve disease in Devic's neuromyelitis optica...
  76. ncbi Immunological phenotyping of fibroblast cultures from patients with a mitochondrial respiratory chain deficit
    S L Williams
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, UK
    Lab Invest 81:1069-77. 2001
    ....
  77. 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...
  78. ncbi Genetic and clinical heterogeneity in paroxysmal kinesigenic dyskinesia: evidence for a third EKD gene
    Sian D Spacey
    Department of Molecular Pathogenesis, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 17:717-25. 2002
    ..This study attempts to clarify the relationship of adolescent and infantile seizures to PKD and provides evidence that PKD is both genetically and clinically heterogeneous...
  79. ncbi Clinical correlates of mitochondrial function in Huntington's disease muscle
    Christopher Turner
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, London, United Kingdom
    Mov Disord 22:1715-21. 2007
    ..These results provide additional evidence that mutant huntingtin influences mitochondrial complex II/III function in non-neuronal tissue (skeletal muscle) and suggest that muscle may be a potential marker of disease progression in HD...
  80. ncbi Etiopathogenesis and treatment of Parkinson's disease
    David A Gallagher
    Department of Clinical Neurosciences, Institute of Neurology, UCL, Rowland Hill Street, London NW3 2PF
    Curr Top Med Chem 9:860-8. 2009
    ..The evidence for treatments of motor complications in PD is discussed as are potential non-dopaminergic therapeutic targets to delay or improve motor complications...
  81. ncbi 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
    ....
  82. ncbi Human complex I defects in neurodegenerative diseases
    A H Schapira
    University Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, Rowland Hill Street, London NW3 2PF, UK
    Biochim Biophys Acta 1364:261-70. 1998
    ..The actions of specific toxins, e.g., MPTP continue to play an important role in our understanding of pathogenesis of neurodegeneration, particularly in PD...
  83. ncbi Mitochondrial disorders
    A H Schapira
    University Department of Clinical Neurosciences, Royal Free and University College School of Medicine, and Institute of Neurology, University College London, UK
    Curr Opin Neurol 13:527-32. 2000
    ..Drugs, toxins and deficiency of nuclear encoded proteins that are targeted at mitochondria are now recognized as important causes of secondary mitochondrial respiratory chain deficiency...
  84. ncbi A clinical and genetic study of SPG5A linked autosomal recessive hereditary spastic paraplegia
    P A Wilkinson
    Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
    Neurology 61:235-8. 2003
    ..84. The locus was refined to a 23.6 cM interval between markers D8S1833 and D8S285. No evidence of oxidative phosphorylation defects was found in muscle biopsies from two affected individuals...
  85. ncbi Assessment of the significance of mitochondrial DNA damage by chemotherapeutic agents
    Soo Lo
    Department of Oncology, Royal Free and University College Medical School, University College London, London W1P 8BT, UK
    Int J Oncol 27:337-44. 2005
    ..Mitochondrial DNA is a critical target for MKT-077 and daunorubicin, and is a potential target for novel chemotherapeutic agents...
  86. ncbi Non-motor symptoms of Parkinson's disease: diagnosis and management
    K Ray Chaudhuri
    Movement Disorders Unit, Kings College Hospital, Guy s King s and St Thomas School of Medicine, London, UK
    Lancet Neurol 5:235-45. 2006
    ..Inevitably, the development of treatments that can slow or prevent the progression of Parkinson's disease and its multicentric neurodegeneration provides the best hope of curing non-motor symptoms...
  87. ncbi Pramipexole protects against apoptotic cell death by non-dopaminergic mechanisms
    Mei Gu
    Royal Free and University College Medical School, University College London, London, UK
    J Neurochem 91:1075-81. 2004
    ..Further evaluation is required to determine whether the neuroprotective action of pramipexole is translated to a disease-modifying effect in PD patients...
  88. ncbi Differences in toxicity of the catechol-O-methyl transferase inhibitors, tolcapone and entacapone to cultured human neuroblastoma cells
    L V Prasad Korlipara
    University Department of Clinical Neuroscience, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Neuropharmacology 46:562-9. 2004
    ..However its toxicity may also involve a mechanism independent of its effects upon oxidative phosphorylation...
  89. ncbi International multicenter pilot study of the first comprehensive self-completed nonmotor symptoms questionnaire for Parkinson's disease: the NMSQuest study
    Kallol Ray Chaudhuri
    Movement Disorders Unit, Kings College Hospital, University Hospital Lewisham, Guy s King s and St Thomas School of Medicine, London, United Kingdom
    Mov Disord 21:916-23. 2006
    ..Furthermore, frequently, problems such as diplopia, dribbling, apathy, blues, taste and smell problems were never previously disclosed to the health professionals...
  90. doi The nondeclaration of nonmotor symptoms of Parkinson's disease to health care professionals: an international study using the nonmotor symptoms questionnaire
    K Ray Chaudhuri
    National Parkinson Foundation Centre of Excellence, Kings College Hospital, Denmark Hill, London, United Kingdom
    Mov Disord 25:704-9. 2010
    ..Use of NMSQuest allows patients to flag symptoms which may be otherwise undeclared and remain untreated when potential treatments exist...
  91. ncbi Mitochondria and amyotrophic lateral sclerosis
    Richard W Orrell
    University Department of Clinical Neurosciences, Royal Free and University College Medical School, London NW3 2PF, United Kingdom
    Int Rev Neurobiol 53:411-26. 2002
  92. ncbi The metric properties of a novel non-motor symptoms scale for Parkinson's disease: Results from an international pilot study
    Kallol Ray Chaudhuri
    National Parkinson Foundation Centre of Excellence, Kings College Hospital, London, United Kingdom
    Mov Disord 22:1901-11. 2007
    ..70). In conclusion, NMSS can be used to assess the frequency and severity of NMS in PD patients across all stages in conjunction with the recently validated non-motor questionnaire...
  93. doi Chaperone-mediated autophagy markers in Parkinson disease brains
    Lydia Alvarez-Erviti
    Centro Investigación Biomédica en Red Enfermedades Neurodegenerativas, University of Navarra, Pamplona, Spain
    Arch Neurol 67:1464-72. 2010
    ..To investigate chaperone-mediated autophagy in the pathogenesis of Parkinson disease (PD)...
  94. doi Friedreich's ataxia impact scale: a new measure striving to provide the flexibility required by today's studies
    Stefan J Cano
    Neurological Outcome Measures Unit, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 24:984-92. 2009
    ..Importantly, all versions can be referred back to the original scale. This study shows one of the many clinical advantages of using Rasch measurement methods to construct rating scales...
  95. ncbi International Cooperative Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich's ataxia?
    Stefan J Cano
    Department of Clinical Neurosciences, Royal Free and University College Medical School, London, UK
    Mov Disord 20:1585-91. 2005
    ..Further validity testing, and examination of responsiveness, is required before the ICARS can be recommended as an outcome measure for treatment trials of FDRA...
  96. ncbi Coordinating outcomes measurement in ataxia research: do some widely used generic rating scales tick the boxes?
    Afsane Riazi
    Neurological Outcomes Measures Unit, Institute of Neurology, London, United Kingdom
    Mov Disord 21:1396-403. 2006
    ..Results highlight the potential limitations of these four scales for evaluating health outcomes in FA and suggest the need for new disease-specific patient-based measures of its impact...
  97. doi Protection against paraquat and A53T alpha-synuclein toxicity by cabergoline is partially mediated by dopamine receptors
    Kai Yin Chau
    University Department of Clinical Neurosciences, Institute of Neurology Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK
    J Neurol Sci 278:44-53. 2009
    ..These results confirm the protective action of cabergoline in reducing cell death in two separate genetic and environmental model systems of PD...
  98. ncbi Rotigotine transdermal patch in early Parkinson's disease: a randomized, double-blind, controlled study versus placebo and ropinirole
    Nir Giladi
    Movement Disorders Unit, Department of Neurology, Tel Aviv Sourasky Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
    Mov Disord 22:2398-404. 2007
    ..Application-site reactions were predominantly mild or moderate in intensity. In conclusion, the rotigotine transdermal patch represents an effective and safe option for the treatment of patients with early Parkinson's disease...
  99. ncbi Role of the pharmacist in the effective management of wearing-off in Parkinson's disease
    William Simonson
    Commission for Certification in Geriatric Pharmacy
    Ann Pharmacother 41:1842-9. 2007
    ..To evaluate the role of the practicing pharmacist in the identification and current treatment of the levodopa wearing-off phenomenon experienced by patients with Parkinson's disease (PD) who are receiving chronic levodopa therapy...
  100. ncbi Neuroprotection for Parkinson's disease: prospects and promises
    C Warren Olanow
    Ann Neurol 53:S1-2. 2003
  101. ncbi Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosis
    Steven R Danielson
    Department of Molecular Biosciences, University of California Davis, School of Veterinary Medicine, Davis, California 95616, USA
    J Biol Chem 277:5810-5. 2002
    ..These data indicate that the most common and severe LHON pathogenic mutations 11778 and 3460 predispose cells to apoptosis, which may be relevant for the pathophysiology of cell death in LHON, and potential therapy...