Research Topics
| A H SchapiraSummaryAffiliation: University College London Country: UK Publications
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Publications
Neuroprotection in Parkinson disease: mysteries, myths, and misconceptionsAnthony 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...- Etiology of Parkinson's diseaseAnthony 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... 
Long-term efficacy and safety of safinamide as add-on therapy in early Parkinson's diseaseA 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)...- Success rate, efficacy, and safety/tolerability of overnight switching from immediate- to extended-release pramipexole in advanced Parkinson's diseaseA 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... - Mitochondrial diseasesAnthony 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... - Rasagiline in neurodegenerationAnthony 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 - The clinical relevance of levodopa toxicity in the treatment of Parkinson's diseaseAnthony 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... - Mitochondrial dysfunction in neurodegenerative diseasesAnthony 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... - Neurobiology and treatment of Parkinson's diseaseAnthony 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... - Drug selection and timing of initiation of treatment in early Parkinson's diseaseAnthony 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... - Molecular and clinical pathways to neuroprotection of dopaminergic drugs in Parkinson diseaseAnthony 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... - Progress in neuroprotection in Parkinson's diseaseA 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... - The scientific and clinical basis for future therapies in Parkinson's diseaseA H V Schapira
University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
Eur J Neurol 15:v. 2008 - Future directions in the treatment of Parkinson's diseaseAnthony 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... - Mitochondria in the aetiology and pathogenesis of Parkinson's diseaseAnthony H V Schapira
University Department of Clinical Neurosciences and Institute of Neurology, University College London, London, UK
Lancet Neurol 7:97-109. 2008.... - Treatment options in the modern management of Parkinson diseaseAnthony H V Schapira
University Department of Clinical Neurosciences, Institute of Neurology, University College London, England
Arch Neurol 64:1083-8. 2007 - Mitochondrial dysfunction in Parkinson's diseaseA 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 - Novel pharmacological targets for the treatment of Parkinson's diseaseAnthony 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.... - 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 
Present and future drug treatment for Parkinson's diseaseA 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...- Levodopa in the treatment of Parkinson's diseaseA 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... - Etiology and pathogenesis of Parkinson diseaseAnthony 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... - Mitochondrial pathology in Parkinson's diseaseAnthony 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... - Aetiopathogenesis of Parkinson's diseaseAnthony 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... - Challenges to the development of disease-modifying therapies in Parkinson's diseaseA 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... - Safinamide in the treatment of Parkinson's diseaseAnthony 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... - Rationale for delayed-start study of pramipexole in Parkinson's disease: the PROUD studyAnthony 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... - Complex I: inhibitors, inhibition and neurodegenerationA 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... - Neuroprotection in Parkinson's diseaseAnthony 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... - Perspectives on recent advances in the understanding and treatment of Parkinson's diseaseA 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... - Molecular and clinical prodrome of Parkinson disease: implications for treatmentAnthony 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... - Future strategies for neuroprotection in Parkinson's diseaseAnthony 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... - 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... - Cytochrome c oxidase deficiency associated with the first stop-codon point mutation in human mtDNAM 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... - Mitochondrial involvement in Parkinson's disease, Huntington's disease, hereditary spastic paraplegia and Friedreich's ataxiaA 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... - Mitochondrial dysfunction in neurodegenerative disordersA 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... - Mitochondrial disordersA 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... - 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... - 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... - HLA class II genotypes in Leber's hereditary optic neuropathyG 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... - 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... - Updated guidelines for the management of Parkinson's diseaseK 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... - Mitochondria and degenerative disordersM Orth
Department of Clinical Neurosciences, Royal Free and University College London Medical School, UK
Am J Med Genet 106:27-36. 2001.... - 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... - 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... - Phenotype, genotype, and worldwide genetic penetrance of LRRK2-associated Parkinson's disease: a case-control studyDaniel 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?.. - Genetic and environmental factors in the cause of Parkinson's diseaseThomas 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... - Silencing of PINK1 expression affects mitochondrial DNA and oxidative phosphorylation in dopaminergic cellsMatthew 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... - Clinical, biochemical and molecular genetic features of Leber's hereditary optic neuropathyR 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... - 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... - Rationale for the use of dopamine agonists as neuroprotective agents in Parkinson's diseaseAnthony 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 - Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagyMatthew 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... - Proteasomal inhibition causes loss of nigral tyrosine hydroxylase neuronsAnthony 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... - Neuroprotection and dopamine agonistsAnthony 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... - Mitochondrial dysfunction associated with neuronal death following status epilepticus in ratHannah 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... - Analysis of mutant DNA polymerase gamma in patients with mitochondrial DNA depletionJan 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... - Relapsing neuropathy in an 18-year-old womanLionel 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 - Analysis of the trinucleotide CAG repeat from the DNA polymerase gamma gene (POLG) in patients with Parkinson's diseaseJan 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... - Disease modification in Parkinson's diseaseAnthony 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... - Polyphenotypic expression of mitochondrial toxicity caused by nucleoside reverse transcriptase inhibitorsRobert 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... - Mitochondrial diseaseAnthony 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... - Restless legs syndrome: an update on treatment optionsAnthony 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... - PINK1-parkin-dependent mitophagy involves ubiquitination of mitofusins 1 and 2: Implications for Parkinson disease pathogenesisMatthew 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... - 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... - Causes of neuronal death in Parkinson's diseaseA 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... - HLA class I genotypes in Leber's hereditary optic neuropathyR 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... - Antioxidant treatment of patients with Friedreich ataxia: four-year follow-upPaul E Hart
University Department of Clinical Neurosciences, Royal Free and University College Medical School, London, England
Arch Neurol 62:621-6. 2005..CONCLUSION: This therapy resulted in sustained improvement in mitochondrial energy synthesis that was associated with a slowing of the progression of certain clinical features and a significant improvement in cardiac function... - Relationship between alpha synuclein phosphorylation, proteasomal inhibition and cell death: relevance to Parkinson's disease pathogenesisKai Yin Chau
University Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
J Neurochem 110:1005-13. 2009.... - Non-motor symptoms of Parkinson's disease: dopaminergic pathophysiology and treatmentK 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... - 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... - Neuromyelitis optica (Devic's syndrome): no association with the primary mitochondrial DNA mutations found in Leber hereditary optic neuropathyH 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... - Metabolic enzyme expression in dopaminergic neurons in Parkinson's disease: an in situ hybridization studyA 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.. - Immunological phenotyping of fibroblast cultures from patients with a mitochondrial respiratory chain deficitS L Williams
University Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, UK
Lab Invest 81:1069-77. 2001.... - 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... - Clinical correlates of mitochondrial function in Huntington's disease muscleChristopher 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... - Etiopathogenesis and treatment of Parkinson's diseaseDavid 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... - Genetic and clinical heterogeneity in paroxysmal kinesigenic dyskinesia: evidence for a third EKD geneSian 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... - 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 disordersA 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... - Human complex I defects in neurodegenerative diseasesA 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... - A clinical and genetic study of SPG5A linked autosomal recessive hereditary spastic paraplegiaP 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... - The nondeclaration of nonmotor symptoms of Parkinson's disease to health care professionals: an international study using the nonmotor symptoms questionnaireK 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... - Pramipexole protects against apoptotic cell death by non-dopaminergic mechanismsMei 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... - Non-motor symptoms of Parkinson's disease: diagnosis and managementK 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... - Differences in toxicity of the catechol-O-methyl transferase inhibitors, tolcapone and entacapone to cultured human neuroblastoma cellsL 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... - Mitochondria and amyotrophic lateral sclerosisRichard 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 - Assessment of the significance of mitochondrial DNA damage by chemotherapeutic agentsSoo 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... - The metric properties of a novel non-motor symptoms scale for Parkinson's disease: Results from an international pilot studyKallol 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... - International multicenter pilot study of the first comprehensive self-completed nonmotor symptoms questionnaire for Parkinson's disease: the NMSQuest studyKallol 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... - Chaperone-mediated autophagy markers in Parkinson disease brainsLydia 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)... - 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... - Friedreich's ataxia impact scale: a new measure striving to provide the flexibility required by today's studiesStefan 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... - 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... - Protection against paraquat and A53T alpha-synuclein toxicity by cabergoline is partially mediated by dopamine receptorsKai 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... - Role of the pharmacist in the effective management of wearing-off in Parkinson's diseaseWilliam 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... - Dopamine agonists in Parkinson's diseaseMitsutoshi Yamamoto
Department of Neurology, Kagawa, Prefectural Central Hospital, Takamatsu, Japan
Expert Rev Neurother 8:671-7. 2008..Thus, dopamine agonists contribute to several dimensions of the management of PD and have become an integral part of the disease treatment algorithm... - Neuroprotection for Parkinson's disease: prospects and promisesC Warren Olanow
Ann Neurol 53:S1-2. 2003 - Rotigotine transdermal patch in early Parkinson's disease: a randomized, double-blind, controlled study versus placebo and ropiniroleNir 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... - Friedreich's ataxia: from disease mechanisms to therapeutic interventionsRaffaele Lodi
Dipartimento di Medicina Clinica e Biotecnologia Applicata, Universita di Bologna, Bologna, Italy
Antioxid Redox Signal 8:438-43. 2006..Pilot studies have shown the potential effect of antioxidant therapy in this condition and provide a strong rationale for designing larger clinical randomized trials... - Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosisSteven 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... - Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose mediumAnna Ghelli
Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Italy
J Biol Chem 278:4145-50. 2003..In conclusion, LHON cybrid cells forced by the reduced rate of glycolytic flux to utilize oxidative metabolism are sensitized to an apoptotic death through a mechanism involving mitochondria...