Kevin Talbot

Summary

Affiliation: University of Oxford
Country: UK

Publications

  1. pmc Development of a patient reported outcome measure for fatigue in motor neurone disease: the Neurological Fatigue Index (NFI-MND)
    Chris J Gibbons
    Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool, UK
    Health Qual Life Outcomes 9:101. 2011
  2. pmc Rasch analysis of the hospital anxiety and depression scale (HADS) for use in motor neurone disease
    Chris J Gibbons
    Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool, UK
    Health Qual Life Outcomes 9:82. 2011
  3. pmc Motor neurone disease
    K Talbot
    Department of Clinical Neurology, Radcliffe Infirmary, Oxford OX2 6HE, UK
    Postgrad Med J 78:513-9. 2002
  4. ncbi request reprint Neuromuscular disorders: therapeutic advances
    Kevin Talbot
    Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford, OX2 6HE, UK
    Lancet Neurol 6:18-9. 2007
  5. ncbi request reprint Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease
    Kevin Talbot
    Department of Physiology, Anatomy and Genetics, University of Oxford, Henry Wellcome Building of Gene Function, South Parks Road, Oxford OX1 3QX, UK
    Hum Mol Genet 15:R182-7. 2006
  6. doi request reprint Is good housekeeping the key to motor neuron survival?
    Kevin Talbot
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK
    Cell 133:572-4. 2008
  7. pmc TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy
    Bradley J Turner
    University of Oxford, MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, South Parks Road, Oxford, OX1 3QX, UK
    BMC Neurosci 9:104. 2008
  8. pmc Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy
    Dirk Bäumer
    MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom
    PLoS Genet 5:e1000773. 2009
  9. doi request reprint Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model
    Bradley J Turner
    MRC Functional Genomics Unit, University of Oxford, Department of Physiology, Anatomy and Genetics, Oxford, UK
    Neurobiol Dis 34:511-7. 2009
  10. ncbi request reprint The molecular genetics of non-ALS motor neuron diseases
    Paul A James
    Department of Physiology, Anatomy and Genetics, Oxford University, Oxford, UK
    Biochim Biophys Acta 1762:986-1000. 2006

Collaborators

Detail Information

Publications46

  1. pmc Development of a patient reported outcome measure for fatigue in motor neurone disease: the Neurological Fatigue Index (NFI-MND)
    Chris J Gibbons
    Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool, UK
    Health Qual Life Outcomes 9:101. 2011
    ..Fatigue was defined as reversible motor weakness and whole-body tiredness that was predominantly brought on by muscular exertion and was partially relieved by rest...
  2. pmc Rasch analysis of the hospital anxiety and depression scale (HADS) for use in motor neurone disease
    Chris J Gibbons
    Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool, UK
    Health Qual Life Outcomes 9:82. 2011
    ..This study seeks to analyse the construct validity of the HADS in MND by fitting its data to the Rasch model...
  3. pmc Motor neurone disease
    K Talbot
    Department of Clinical Neurology, Radcliffe Infirmary, Oxford OX2 6HE, UK
    Postgrad Med J 78:513-9. 2002
    ..If, as seems likely, complex inherited and environmental factors contribute to the pathogenesis of MND, future treatment may involve a combination of molecular based treatments or restoration of cellular integrity using stem cell grafts...
  4. ncbi request reprint Neuromuscular disorders: therapeutic advances
    Kevin Talbot
    Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford, OX2 6HE, UK
    Lancet Neurol 6:18-9. 2007
  5. ncbi request reprint Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease
    Kevin Talbot
    Department of Physiology, Anatomy and Genetics, University of Oxford, Henry Wellcome Building of Gene Function, South Parks Road, Oxford OX1 3QX, UK
    Hum Mol Genet 15:R182-7. 2006
    ..Pure ALS, through ALS with cognitive impairment and ALS-FTD to pure FTD-U, may represent a continuous spectrum of ubiquitin-associated neurodegenerative disease...
  6. doi request reprint Is good housekeeping the key to motor neuron survival?
    Kevin Talbot
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK
    Cell 133:572-4. 2008
    ..Reporting in this issue, Zhang et al. (2008) challenge prior assumptions about the housekeeping function of SMN and demonstrate that loss of SMN leads to highly tissue-specific effects on splicing...
  7. pmc TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy
    Bradley J Turner
    University of Oxford, MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, South Parks Road, Oxford, OX1 3QX, UK
    BMC Neurosci 9:104. 2008
    ..Here, we characterise TDP-43 localisation, expression levels and post-translational modifications in mouse models of ALS and spinal muscular atrophy (SMA)...
  8. pmc Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy
    Dirk Bäumer
    MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom
    PLoS Genet 5:e1000773. 2009
    ....
  9. doi request reprint Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model
    Bradley J Turner
    MRC Functional Genomics Unit, University of Oxford, Department of Physiology, Anatomy and Genetics, Oxford, UK
    Neurobiol Dis 34:511-7. 2009
    ..We therefore propose that SMN replacement and upregulation strategies considered for SMA therapy may have protective potential for ALS...
  10. ncbi request reprint The molecular genetics of non-ALS motor neuron diseases
    Paul A James
    Department of Physiology, Anatomy and Genetics, Oxford University, Oxford, UK
    Biochim Biophys Acta 1762:986-1000. 2006
    ..We review the clinical and molecular features of this diverse group of genetically determined conditions and consider the implications for the broad group of motor neuron diseases in general...
  11. doi request reprint Dismutase-competent SOD1 mutant accumulation in myelinating Schwann cells is not detrimental to normal or transgenic ALS model mice
    Bradley J Turner
    MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, John Radcliffe Hospital, University of Oxford, Oxford OX1 3QX, UK
    Hum Mol Genet 19:815-24. 2010
    ..We conclude that dismutase-competent mutant SOD1 accumulation within Schwann cells is not pathological to spinal motor neurons or deleterious to disease course in transgenic ALS model mice, in contrast to astrocytes and microglia...
  12. pmc T2-weighted MRI detects presymptomatic pathology in the SOD1 mouse model of ALS
    Matthew C Evans
    1 Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK 2 CR UK MRC Gray Institute for Radiation Oncology and Biology, Department of Oncology, University of Oxford, Oxford, UK
    J Cereb Blood Flow Metab 34:785-93. 2014
    ....
  13. pmc Autoimmune disease preceding amyotrophic lateral sclerosis: an epidemiologic study
    Martin R Turner
    From the Oxford University Nuffield Department of Clinical Neurosciences M R T, K T, John Radcliffe Hospital Oxford University Unit of Health Care Epidemiology R G, M J G, Department of Public Health and Oxford University Department of Physiology, Anatomy and Genetics S R, Oxford, UK
    Neurology 81:1222-5. 2013
    ..To study whether the risk of amyotrophic lateral sclerosis (ALS) is increased in people with prior autoimmune disease...
  14. ncbi request reprint The role of RNA processing in the pathogenesis of motor neuron degeneration
    Dirk Bäumer
    MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3QX, UK
    Expert Rev Mol Med 12:e21. 2010
    ..Complete understanding of how these pathways interact and elucidation of specialised mechanisms for mRNA targeting and processing in motor neurons are likely to produce new targets for therapy in ALS and related disorders...
  15. doi request reprint Cardiovascular fitness as a risk factor for amyotrophic lateral sclerosis: indirect evidence from record linkage study
    Martin R Turner
    Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK
    J Neurol Neurosurg Psychiatry 83:395-8. 2012
    ..Hospital admission for coronary heart disease (CHD) might serve as an objective marker of reduced cardiovascular fitness...
  16. pmc The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy
    James N Sleigh
    MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, South Parks Road, Oxford, OX1 3QX, UK
    Dis Model Mech 4:457-67. 2011
    ....
  17. pmc Pattern of spread and prognosis in lower limb-onset ALS
    Martin R Turner
    Oxford University Department of Clinical Neurology, Oxford, UK
    Amyotroph Lateral Scler 11:369-73. 2010
    ..The time interval to this initial spread is a powerful factor in predicting overall survival, and could be used to facilitate decision-making and effective care planning...
  18. pmc Whole-brain magnetic resonance spectroscopic imaging measures are related to disability in ALS
    Charlotte J Stagg
    Centre for Functional Magnetic Resonance of the Brain, University of Oxford, UK
    Neurology 80:610-5. 2013
    ....
  19. ncbi request reprint The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis
    Martin R Turner
    Oxford University Department of Clinical Neurology, John Radcliffe Hospital, Oxford, UK
    J Neurol Sci 294:81-5. 2010
    ..Diagnostic delay is a common occurrence in ALS, and many BO patients report having attended other specialist clinics prior to diagnosis...
  20. doi request reprint Biomarkers in amyotrophic lateral sclerosis
    Martin R Turner
    Department of Clinical Neurology, University of Oxford, Oxford, UK
    Lancet Neurol 8:94-109. 2009
    ..Such biomarkers might also resolve complexities of phenotypic heterogeneity in clinical trials. In this Review, we discuss the development of biomarkers in ALS and consider potential future directions for research...
  21. ncbi request reprint A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes
    Steven Ackerley
    Department of Human Anatomy and Genetics, South Parks Road, Oxford OX1 3QX, UK
    Hum Mol Genet 15:347-54. 2006
    ..These findings suggest a possible pathogenic mechanism for HSPB1 whereby the mutation may lead to preferential motor neuron loss by disrupting selective components essential for axonal structure and transport...
  22. doi request reprint Age-dependent and -independent behavioral deficits in Tg2576 mice
    R M J Deacon
    Department of Experimental Psychology, University of Oxford, South Parks Road, Oxford OX1 3UD, UK
    Behav Brain Res 189:126-38. 2008
    ..However, this model might perform better behaviorally on a different genetic background...
  23. pmc REM sleep behaviour disorder is associated with worse quality of life and other non-motor features in early Parkinson's disease
    Michal Rolinski
    Department of Physiology, Anatomy and Genetics, Oxford Parkinson s Disease Centre, Oxford, UK
    J Neurol Neurosurg Psychiatry 85:560-6. 2014
    ..Although the brainstem structures responsible for the symptoms of RBD correspond to the premotor stages of PD, the association of RBD with motor and non-motor features in early PD remains unclear...
  24. doi request reprint Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy
    James N Sleigh
    Department of Physiology, Anatomy and Genetics, MRC Functional Genomics Unit, University of Oxford, South Parks Road, Oxford OX1 3PT, UK
    Hum Mol Genet 23:855-69. 2014
    ..Our findings thus link the dysregulation of Chodl to the pathophysiology of motor neuron degeneration in SMA. ..
  25. pmc Mimics and chameleons in motor neurone disease
    Martin R Turner
    University of Oxford Nuffield Department of Clinical Neurosciences, Oxford, UK
    Pract Neurol 13:153-64. 2013
    ....
  26. doi request reprint Magnetic resonance imaging of pathological processes in rodent models of amyotrophic lateral sclerosis
    Matthew C Evans
    Oxford University Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK
    Amyotroph Lateral Scler 13:288-301. 2012
    ..These are potentially important steps towards the ultimate goal of human therapeutic translation...
  27. doi request reprint HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes
    Alice S Kwok
    MRC Functional Genomics Unit, Department of Anatomy Physiology and Genetics, University of Oxford, South Parks Road, Oxford, UK
    J Neurochem 119:1155-61. 2011
    ..We conclude that defects in HspB8-mediated autophagy are likely to contribute to dHMNII pathology and their detection in peripheral blood mononuclear cells could be a useful, accessible biomarker for the disease...
  28. doi request reprint Integration of structural and functional magnetic resonance imaging in amyotrophic lateral sclerosis
    Gwenaelle Douaud
    Oxford Centre for Functional Magnetic Resonance of the Brain FMRIB, University of Oxford, John Radcliffe Hospital, Oxford, OX3 9DU, UK
    Brain 134:3470-9. 2011
    ....
  29. doi request reprint Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS
    Bradley J Turner
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK
    Prog Neurobiol 85:94-134. 2008
    ..This review summarises the wealth of known genetic and therapeutic modifiers in rodent models with SOD1 mutations and discusses these in the wider context of ALS pathoetiology and treatment...
  30. ncbi request reprint Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy
    Muhammed Z Cader
    Henry Wellcome Building for Gene Function, MRC Functional Genetics Unit, University of Oxford, South Parks Road, Oxford OX1 3QX, United Kingdom
    FEBS Lett 581:2959-64. 2007
    ..Reduced enzyme activity may underlie neuronal degeneration, although a dominant-negative effect is more likely in this autosomal dominant disorder...
  31. doi request reprint Head and other physical trauma requiring hospitalisation is not a significant risk factor in the development of ALS
    Martin R Turner
    Oxford University Department of Clinical Neurology, John Radcliffe Hospital, Oxford, OX3 9DU, UK
    J Neurol Sci 288:45-8. 2010
    ..The high risk of head injury observed in the immediate post-diagnosis period may be amenable to primary prevention...
  32. doi request reprint Oculomotor dysfunction in amyotrophic lateral sclerosis: a comprehensive review
    Rakesh Sharma
    Oxford University Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, United Kingdom
    Arch Neurol 68:857-61. 2011
    ..The study of oculomotor dysfunction holds significant promise as an additional source of much needed prognostic, monitoring, and mechanistic biomarkers for ALS...
  33. ncbi request reprint Spinal muscular atrophies reveal motor neuron vulnerability to defects in ribonucleoprotein handling
    Kirstie Anderson
    Department of Human Anatomy and Genetics, University of Oxford and Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK
    Curr Opin Neurol 16:595-9. 2003
    ....
  34. pmc An eye-tracking version of the trail-making test
    Stephen L Hicks
    Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom
    PLoS ONE 8:e84061. 2013
    ..There was good correlation for speed between both versions of Part B (R(2)=0.73), suggesting that this is a viable method to objectively assess cognitive impairment in disorders where patients are unable to speak or write. ..
  35. pmc Amyotrophic lateral sclerosis: cell vulnerability or system vulnerability?
    Kevin Talbot
    Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK
    J Anat 224:45-51. 2014
    ....
  36. doi request reprint Comprehensive morphometry of subcortical grey matter structures in early-stage Parkinson's disease
    Ricarda A L Menke
    FMRIB Centre, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, United Kingdom Department of Psychiatry, Warneford Hospital, University of Oxford, Oxford, United Kingdom Oxford Parkinson s Disease Centre OPDC, University of Oxford, Oxford, United Kingdom
    Hum Brain Mapp 35:1681-90. 2014
    ..However, the subtle nature of these changes makes it unlikely that shape analysis alone will be useful for early diagnosis...
  37. ncbi request reprint Controversies and priorities in amyotrophic lateral sclerosis
    Martin R Turner
    Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
    Lancet Neurol 12:310-22. 2013
    ....
  38. doi request reprint Functional vitamin B12 deficiency
    Martin R Turner
    Department of Neurology, John Radcliffe Hospital, University of Oxford, Oxford, UK
    Pract Neurol 9:37-41. 2009
    ..Such patients may respond to repeated high-dose injections of B12...
  39. ncbi request reprint Activation of mutant protein kinase Cgamma leads to aberrant sequestration and impairment of its cellular function
    Graeme Doran
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, South Parks Road, Oxford OX1 3QX, UK
    Biochem Biophys Res Commun 372:447-53. 2008
    ..These studies suggest that Purkinje cell damage in SCA14 may result from a reduction of PKCgamma activity due its aberrant sequestration in the early endosome compartment...
  40. ncbi request reprint A case of celiac disease mimicking amyotrophic lateral sclerosis
    Martin R Turner
    Department of Neurology, John Radcliffe Hospital, Oxford, UK
    Nat Clin Pract Neurol 3:581-4. 2007
    ..The thigh muscle in the affected leg showed signs of wasting. The patient had a remote family history of celiac disease...
  41. doi request reprint Candidate screening of the bovine and feline spinal muscular atrophy genes reveals no evidence for involvement in human motor neuron disorders
    N J Parkinson
    University of Oxford, MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, Henry Wellcome Centre for Gene Function, South Parks Road, Oxford OX1 3QX, UK
    Neuromuscul Disord 18:394-7. 2008
    ..This study indicates that mutations in these genes do not contribute significantly to the cause of motor neuron diseases in the human population...
  42. doi request reprint Asymmetrical late onset motor neuropathy associated with a novel mutation in the small heat shock protein HSPB1 (HSP27)
    P A James
    MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, UK
    J Neurol Neurosurg Psychiatry 79:461-3. 2008
    ..Expression of this and other known mutations in cell culture demonstrated enhanced aggregation of mutant HSPB1 protein compared with wild-type...
  43. ncbi request reprint Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy
    Lyndsay M Murray
    Centre for Integrative Physiology, University of Edinburgh Medical School, Edinburgh EH8 9XD, UK
    Hum Mol Genet 17:949-62. 2008
    ....
  44. ncbi request reprint Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy
    Oleg V Evgrafov
    Department of Psychiatry, New York State Psychiatric Institute Research Foundation for Mental Hygiene, Unit 28, 1051 Riverside Drive, New York, New York 10032, USA
    Nat Genet 36:602-6. 2004
    ..Cotransfection of neurofilament light chain (NEFL) and mutant HSPB1 resulted in altered neurofilament assembly in cells devoid of cytoplasmic intermediate filaments...
  45. ncbi request reprint Musculoskeletal diseases: from complex genetics to therapy
    Kevin Talbot
    Curr Opin Pharmacol 3:277-9. 2003
  46. ncbi request reprint The study of rare diseases: butterfly collecting or an entrée to understanding common conditions?
    Kevin Talbot
    Pract Neurol 7:210-1. 2007