Research Topics
Genomes and Genes | Angela VincentSummaryAffiliation: University of Oxford Country: UK Publications
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Publications
Antibodies associated with paraneoplastic neurological disordersA Vincent
University of Oxford, John Radcliffe Hospital, Oxford OX3 9DS, UK
Neurol Sci 26:S3-4. 2005..There are also some ELISA assays available for detection of antibodies to specific antigens...
Immunology of disorders of neuromuscular transmissionA Vincent
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
Acta Neurol Scand Suppl 183:1-7. 2006..The pathogenic mechanisms probably depend on the IgG subclass of the antibodies and are only partly shared between the diseases...
The growing recognition of immunotherapy-responsive seizure disorders with autoantibodies to specific neuronal proteinsAngela Vincent
Department of Clinical Neurology, John Radcliffe Hospital, Oxford, UK
Curr Opin Neurol 23:144-50. 2010..The concept of epilepsy and seizure disorders caused by autoantibodies to specific neuronal membrane proteins has developed significantly during the past few years...
Maternal antibody-mediated dyslexia? Evidence for a pathogenic serum factor in a mother of two dyslexic children shown by transfer to mice using behavioural studies and magnetic resonance spectroscopyAngela Vincent
Neurosciences Group, Department of Clinical Neurology, Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, OX3 9DS, Oxford, UK
J Neuroimmunol 130:243-7. 2002..These preliminary results are consistent with a role for maternal serum factors, probably antibodies, in causing some of the features of dyslexia, and possibly in other neurodevelopmental disorders...
Systemic and neurologic autoimmune disorders associated with seizures or epilepsyAngela Vincent
Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, Oxford, United Kingdom
Epilepsia 52:12-7. 2011....
Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitisAngela Vincent
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford OX3 9DS, UK
Brain 127:701-12. 2004..Establishing the frequency of this new syndrome, the full range of clinical presentations and means of early recognition, and optimal immunotherapy, should now be the aim...
Antibodies in myasthenia gravis and related disordersAngela Vincent
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, United Kingdom
Ann N Y Acad Sci 998:324-35. 2003..These antibodies are not found in AChR antibody-positive MG and are predominantly IgG4. MuSK antibody positivity appears to be associated with more severe bulbar disease that can be difficult to treat effectively...
Autoimmune disorders of the neuromuscular junctionAngela Vincent
Department of Clinical Neurology, Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, United Kingdom
Neurol India 56:305-13. 2008..The immune mechanisms of acquired NMT relate to loss of voltage-gated potassium channel function. This review will focus on the important recent developments in the immune-mediated disorders of the NMJ...
Caspr2 antibodies in patients with thymomasAngela Vincent
Department of Clinical Neurology, John Radcliffe Hospital, Oxford, United Kingdom
J Thorac Oncol 5:S277-80. 2010..These antibodies are common in the subgroup of patients with thymic malignancies...
Evidence of underdiagnosis of myasthenia gravis in older peopleA Vincent
Department of Clinical Neurology and Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DS, UK
J Neurol Neurosurg Psychiatry 74:1105-8. 2003..There is anecdotal evidence that the diagnosis is sometimes missed in older patients...
Seronegative generalised myasthenia gravis: clinical features, antibodies, and their targetsAngela Vincent
Department of Clinical Neurology, University of Oxford, UK
Lancet Neurol 2:99-106. 2003..Moreover, muscle wasting may be present, which prevents complete response to these therapies...
Autoimmune channelopathies: well-established and emerging immunotherapy-responsive diseases of the peripheral and central nervous systemsAngela Vincent
Department of Clinical Neurology, Level 6 West Wing, John Radcliffe Hospital, University of Oxford, Oxford, UK
J Clin Immunol 30:S97-102. 2010....
Unravelling the pathogenesis of myasthenia gravisAngela Vincent
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, UK
Nat Rev Immunol 2:797-804. 2002....
Myasthenia gravisAngela Vincent
Neurosciences Group and Stroke Prevention Group, Department of Clinical Neurology, University of Oxford, UK
Autoimmunity 37:317-9. 2004
Molecular targets for autoimmune and genetic disorders of neuromuscular transmissionA Vincent
Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
Eur J Biochem 267:6717-28. 2000..Study of these conditions has greatly increased our understanding of synaptic function and of disease aetiology...
Pathogenic autoantibodies to neuronal proteins in neurological disordersA Vincent
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
J Neuroimmunol 100:169-80. 1999....
Autoimmune channelopathies and related neurological disordersAngela Vincent
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, United Kingdom
Neuron 52:123-38. 2006....
Antibody-mediated disorders of neuromuscular transmissionAngela Vincent
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, UK
Suppl Clin Neurophysiol 57:147-58. 2004
Determinant spreading and immune responses to acetylcholine receptors in myasthenia gravisA Vincent
Department of Clinical Neurology, John Radcliffe Hospital, Oxford, UK
Immunol Rev 164:157-68. 1998..Various possibilities, including upregulation of class II on muscle/myoid cells and involvement of CD8+ responses to AChR and other muscle antigens, are discussed...
Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravisAngela Vincent
Neurosciences Group, Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, UK
Curr Opin Neurol 18:519-25. 2005..Here we review recent clinical and experimental studies on MuSK antibody associated myasthenia gravis, and summarize the results of newer treatments for myasthenia gravis...
Myasthenia gravis seronegative for acetylcholine receptor antibodiesAngela Vincent
Neurosciences Group, Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, John Radcliffe Hospital, Oxford, United Kingdom
Ann N Y Acad Sci 1132:84-92. 2008..Overall, these results suggest that complement-activation may be an important pathogenic mechanism even in patients without conventional AChR antibodies...
Immunology of the neuromuscular junction and presynaptic nerve terminalA Vincent
University of Oxford, Department of Clinical Neurology, John Radcliffe Hospital, UK
Curr Opin Neurol 12:545-51. 1999....
Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotoniaSarosh R Irani
Neurosciences Group, Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital, Oxford, UK
Brain 133:2734-48. 2010....
IgG1 antibodies to acetylcholine receptors in 'seronegative' myasthenia gravisMaria Isabel Leite
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford OX3 9DS, UK
Brain 131:1940-52. 2008..These observations throw new light on different forms of MG paving the way for improved diagnosis and management, and the approaches used have applicability to other antibody-mediated conditions...
Morvan syndrome: clinical and serological observations in 29 casesSarosh R Irani
Neurosciences Group, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, United Kingdom
Ann Neurol 72:241-55. 2012....
Dok-7 mutations underlie a neuromuscular junction synaptopathyDavid Beeson
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford OX3 9DS, UK
Science 313:1975-8. 2006..We showed that recessive inheritance of mutations in Dok-7, which result in a defective structure of the neuromuscular junction, is a cause of CMS with proximal muscle weakness...
New autoantibody mediated disorders of the central nervous systemBethan Lang
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, University of Oxford, UK
Curr Opin Neurol 16:351-7. 2003..This review summarizes the recent findings and assesses the evidence that these conditions are caused by the antibodies, using the criteria established for peripheral nervous system autoimmune diseases...
Diverse molecular mechanisms involved in AChR deficiency due to rapsyn mutationsJudy Cossins
Neurosciences Group, Weatherall Institute of Molecular Medicine, Churchill Hospital, Oxford, UK
Brain 129:2773-83. 2006..The disease severity of patients harbouring the compound allelic mutations was greater than that of patients with homozygous rapsyn mutation N88K, suggesting that the second mutant allele may largely determine severity...
Non-radioactive serological diagnosis of myasthenia gravis and clinical features of patients from Tianjin, ChinaLi Yang
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, England, UK
J Neurol Sci 301:71-6. 2011..To establish non-radioactive assays for detection of antibodies (Abs) to the acetylcholine receptor (AChR) and to muscle specific kinase (MuSK). To show that the assays can be used in Tianjin for testing patients with MG...
Passive and active immunization models of MuSK-Ab positive myasthenia: electrophysiological evidence for pre and postsynaptic defectsStuart Viegas
Weatherall Institute of Molecular Medicine and Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford OX3 9DS, UK
Exp Neurol 234:506-12. 2012..These results raise questions about how MuSK is involved in retrograde signaling, and the combination of post-synaptic defects with lack of presynaptic compensation may begin to explain the more severe disease in MuSK-MG patients...
Structural abnormalities of the AChR caused by mutations underlying congenital myasthenic syndromesDavid Beeson
Neurosciences Group, Weatherall Institute of Molecular Medicine, The John Radcliffe, Headington, Oxford OX3 9DS, United Kingdom
Ann N Y Acad Sci 998:114-24. 2003..It was found that mutations within muscle AChRs are the most common cause of CMS. The majority are located within the epsilon-subunit gene and result in AChR deficiency...
Effect of sera from AChR-antibody negative myasthenia gravis patients on AChR and MuSK in cell culturesMaria Elena Farrugia
Neurosciences Group, Weatherall Institute of Molecular Medicine, Department of Clinical Neurology, University of Oxford, UK
J Neuroimmunol 185:136-44. 2007..Thus, although these results have, disappointingly, demonstrated little effect of MuSK antibodies on AChR expression, they do imply that SNMG antibodies act on AChR-associated pathways...
Spontaneous production of anti-IFN-alpha and anti-IL-12 autoantibodies by thymoma cells from myasthenia gravis patients suggests autoimmunization in the tumorHiroyuki Shiono
Neuroscience Group, Weatherall Institute for Molecular Medicine, University of Oxford, Oxford OX3 9DS, UK
Int Immunol 15:903-13. 2003..With these molecules, it should be easier to identify provoking cell type(s) that may give novel additional clues to autoimmunization against T-cell epitopes from the more complex AChR...
MRI and clinical studies of facial and bulbar muscle involvement in MuSK antibody-associated myasthenia gravisMaria Elena Farrugia
Department of Clinical Neurology, University of Oxford, Oxford, UK
Brain 129:1481-92. 2006....
MuSK antibody positive myasthenia gravis plasma modifies MURF-1 expression in C2C12 cultures and mouse muscle in vivoOlivier Benveniste
Department of Clinical Neurology, University of Oxford OX3 9DS, UK
J Neuroimmunol 170:41-8. 2005..They indicate the need for further studies on muscle atrophy, MuSK-MG antibodies, the effects of steroids, and the intracellular pathways involved...
Presence and pathogenic relevance of antibodies to clustered acetylcholine receptor in ocular and generalized myasthenia gravisSaiju Jacob
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, United Kingdom
Arch Neurol 69:994-1001. 2012....
Congenital myasthenic syndromes and the formation of the neuromuscular junctionDavid Beeson
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK
Ann N Y Acad Sci 1132:99-103. 2008....
Single-fiber electromyography in limb and facial muscles in muscle-specific kinase antibody and acetylcholine receptor antibody myasthenia gravisMaria Elena Farrugia
Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford, UK
Muscle Nerve 33:568-70. 2006..These findings demonstrate clear differences between the neurophysiology of MuSK-MG and AChR-MG...
Antibodies to neuronal targets in neurological and psychiatric diseasesAngela Vincent
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
Ann N Y Acad Sci 992:48-55. 2003..This review highlights features of these conditions, preliminary investigations into neurodevelopmental disorders, and areas for further study...
Autoimmune disorders of neuronal potassium channelsJohn Newsom-Davis
Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
Ann N Y Acad Sci 998:202-10. 2003....
Lack of association between acetylcholine receptor epsilon polymorphisms and early-onset myasthenia gravisDomenico Marco Bonifati
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, United Kingdom
Muscle Nerve 29:436-9. 2004..These data provide no evidence that heteroallelic mutations or polymorphisms in the AChR epsilon subunit are involved in the development of autoimmune early-onset MG but raise issues for future studies...
Preferential expression of AChR epsilon-subunit in thymomas from patients with myasthenia gravisCalman A MacLennan
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford OX3 9DS, UK
J Neuroimmunol 201:28-32. 2008..The greater abundance of mRNA for AChR epsilon-subunit than for other subunits suggests that the AChR epsilon-subunit may play a distinctive role in autosensitization in MG-associated thymomas, particularly those of type A or AB...
Autoimmunizing mechanisms in thymoma and thymusNick Willcox
Neuroscience Group, Weatherall Institute for Molecular Medicine, University of Oxford, England, UK
Ann N Y Acad Sci 1132:163-73. 2008..Together, moreover, our findings implicate both myoid cells and TEC in autoimmunization, and thus strongly support our hypothesis...
Pathogenic autoantibodies in the lambert-eaton myasthenic syndromeBethan Lang
Neurosciences Group and Department of Clinical Neurology, Oxford University, Oxford, United Kingdom
Ann N Y Acad Sci 998:187-95. 2003..These results demonstrate an unexpected plasticity in the expression of VGCCs in mammalian neurons and may represent a mechanism by which the pathogenic effects of LEMS IgG are reduced...
The search for new antigenic targets in myasthenia gravisJudith Cossins
Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom
Ann N Y Acad Sci 1275:123-8. 2012..These assays, combined with use of myotube cultures to explore the effects of the antibodies, enable us to begin to identify new antigenic targets and test antibody pathogenicity in vitro...
Contactin-associated protein-2 antibodies in non-paraneoplastic cerebellar ataxiaEsther B E Becker
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford University, Oxford, UK
J Neurol Neurosurg Psychiatry 83:437-40. 2012..Relatively few studies have searched for potentially pathogenic antibodies in non-paraneoplastic patients with cerebellar ataxia...
Inhibition of acetylcholine receptor function by seronegative myasthenia gravis non-IgG factor correlates with desensitisationIan Spreadbury
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, United Kingdom
J Neuroimmunol 162:149-56. 2005..The results suggest that, rather than acting indirectly as previously proposed, the SNMG factor may bind directly to an allosteric site that induces or enhances AChR desensitisation...
Frequency and prognostic impact of antibodies to aquaporin-4 in patients with optic neuritisSven Jarius
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK
J Neurol Sci 298:158-62. 2010....
Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitisSarosh R Irani
Department of Clinical Neurology, Oxford University, Oxford, UK
Ann Neurol 69:892-900. 2011..To describe a distinctive seizure semiology that closely associates with voltage-gated potassium channel (VGKC)-complex/Lgi1 antibodies and commonly precedes the onset of limbic encephalitis (LE)...
Antibodies to acetylcholine receptor in parous women with myasthenia: evidence for immunization by fetal antigenIan Matthews
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, United Kingdom
Lab Invest 82:1407-17. 2002....
Immunocapture and identification of cell membrane protein antigenic targets of serum autoantibodiesEdward Littleton
Weatherall Institute of Molecular Medicine, Oxford, United Kingdom
Mol Cell Proteomics 8:1688-96. 2009..The approach would also be very suitable for the analysis of human or experimental antitumor responses...
The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathiesSarosh R Irani
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK
Arq Neuropsiquiatr 70:300-4. 2012..In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field...
Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and JapanJoanna Kitley
Nuffield Department of Clinical Neurosciences, West Wing, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DU, UK
Brain 135:1834-49. 2012..These factors need to be explored further in future prospective neuromyelitis optica and neuromyelitis optica spectrum disorder studies...
Seronegative myasthenia gravisAngela Vincent
Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
Semin Neurol 24:125-33. 2004..MuSK antibody-positive patients may also differ in the distribution of their electrophysiological abnormalities and their responses to treatments...
Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis opticaSven Jarius
Weatherall Institute of Molecular Medicine, University of Oxford, UK
Nat Clin Pract Neurol 4:202-14. 2008..Many aspects of the pathogenesis, however, remain unclear...
Autoantibodies to glutamic acid decarboxylase in patients with epilepsy are associated with low cortical GABA levelsCharlotte J Stagg
Centre for Functional Magnetic Resonance of the Brain FMRIB, University of Oxford, Oxford, United Kingdom
Epilepsia 51:1898-901. 2010....
Diagnostic use of autoantibodies in myasthenia gravisM Isabel Leite
Department of Clinical Neurology, John Radcliffe Hospital, University of Oxford, West Wing OX3 9DS, UK
Autoimmunity 43:371-9. 2010..The same approaches to antibody detection are now being applied to a wide range of other autoantigens and other autoimmune diseases...
N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexesSarosh R Irani
Department of Clinical Neurology, L6 West Wing, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DU, UK
Brain 133:1655-67. 2010..Four patients, who only had temporal lobe epilepsy without oligoclonal bands, may represent restriction to the first stage...
Acid-sensing ion channel-1 contributes to axonal degeneration in autoimmune inflammation of the central nervous systemManuel A Friese
Medical Research Council Human Immunology Unit and Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DS, UK
Nat Med 13:1483-9. 2007..In addition, adoptive transfer of T cells from wild-type mice did not affect the protection mediated by Asic1 disruption. These results suggest that ASIC1 blockers could provide neuroprotection in multiple sclerosis...
Fetal arthrogryposis and maternal serum antibodiesPaola Dalton
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DS, UK
Neuromuscul Disord 16:481-91. 2006..Other maternal antibodies require further study, but these observations add to the emerging literature on maternal antibodies associated with developmental intrauterine disorders...
Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody statusMaria I Leite
Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
Am J Pathol 171:893-905. 2007..Remarkably, the similar changes place many apparent AChR-seronegative patients in the same spectrum as the AChR-seropositive patients...
NMDA receptor antibody encephalitisSarosh R Irani
Nuffield Department of Clinical Neurosciences, Level 6, West Wing, John Radcliffe Hospital, Headley Way, Oxford OX39DS, UK
Curr Neurol Neurosci Rep 11:298-304. 2011....
Prospective study into the incidence of Lambert Eaton myasthenic syndrome in small cell lung cancerMiranda Payne
Department of Medical Oncology, University of Oxford Medical Oncology Unit, Oxford, United Kingdom
J Thorac Oncol 5:34-8. 2010..VGCC can also be found in patients with SCLC and cerebellar ataxia. This was a prospective study to assess the incidence of clinical and subclinical LEMS or other neurologic disease in patients with SCLC...
Serum autoantibodies to cell surface determinants in multiple sclerosis: a flow cytometric studyOliver Lily
Neurosciences Group, Weatherall Institute of Molecular Medicine, Department of Clinical Neurology, University of Oxford, UK
Brain 127:269-79. 2004..Identification of the cell surface determinants to which the antibodies bind may shed light on new targets for therapeutic intervention...
Detection and characterization of MuSK antibodies in seronegative myasthenia gravisJohn McConville
Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, Oxford, UK
Ann Neurol 55:580-4. 2004..They bind to the extracellular Ig-like domains of soluble or native MuSK. Surprisingly they are predominantly in the IgG4 subclass. MuSK-antibody associated MG may be different in etiological and pathological mechanisms...
Autoimmune disorders of the neuromuscular junctionBethan Lang
Department of Clinical Neurology, University of Oxford, Oxford OX3 9DS, UK
Curr Opin Pharmacol 9:336-40. 2009..General immunosuppression is still the main treatment, but novel treatments that reduce complement-mediated damage or inhibit the binding of pathogenic antibodies are beginning to look promising...
Autoantibodies to ion channels at the neuromuscular junctionBethan Lang
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK
Autoimmun Rev 2:94-100. 2003....
Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitisPatrick Waters
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, England
Arch Neurol 65:913-9. 2008..There is increasing recognition of antibody-mediated immunotherapy-responsive neurologic diseases and a need for appropriate immunoassays...
Maternal neuronal antibodies associated with autism and a language disorderPaola Dalton
Neurosciences Group, Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
Ann Neurol 53:533-7. 2003..This evidence supports a role for maternal antibodies in some forms of neurodevelopmental disorder...
Fewer thymic changes in MuSK antibody-positive than in MuSK antibody-negative MGMaria Isabel Leite
Neurosciences Group, Department of Clinical Neurology, University of Oxford, Oxford OX3 9DU, United Kingdom
Ann Neurol 57:444-8. 2005..However, approximately 75% MuSK- samples showed lymph node-type infiltrates similar to those in AChR+ patients, but with fewer germinal centers. These variations may explain some apparent differences in responses to thymectomy in SNMG...
Detection of anti-aquaporin-4 antibodies in neuromyelitis optica: current status of the assaysP Waters
Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK
Int MS J 15:99-105. 2008..Since the discovery of a specific serum marker for NMO in 2004, and its subsequent identification as an antibody to aquaporin-4 (AQP-4), various methods have been developed to test for the antibodies in patients sera...
Scenarios for autoimmunization of T and B cells in myasthenia gravisH Shiono
Neuroscience Group, Weatherall Institute for Molecular Medicine, University of Oxford, Oxford OX3 9DS, United Kingdom
Ann N Y Acad Sci 998:237-56. 2003..Both these and the consequently enhanced heterogeneity and pathogenicity of the autoantibodies should be minimized by early thymectomy...
Presentation by myoblasts of an epitope from endogenous acetylcholine receptor indicates a potential role in the spreading of the immune responseJ Curnow
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, OX3 9DS, Oxford, UK
J Neuroimmunol 115:127-34. 2001....
Myasthenia gravis in a woman with congenital AChR deficiency due to epsilon-subunit mutationsRebecca Croxen
Neurosciences Group, Weatherall Institute of Molecular Medicine, The John Radcliffe, Oxford, United Kingdom
Neurology 58:1563-5. 2002..The younger sister developed MG at 34 years. This unusual case raises the possibility that genetic defects of the AChR might be a factor in the etiology of autoimmune MG...
The agrin/muscle-specific kinase pathway: new targets for autoimmune and genetic disorders at the neuromuscular junctionYohan Liyanage
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK
Muscle Nerve 25:4-16. 2002..We then review the development of the NMJ, focusing on the important roles of nerve-derived agrin and MuSK in clustering of AChRs and other essential components of the NMJ...
Autoimmune channelopathies: John Newsom-Davis's work and legacy. A summary of the Newsom-Davis Memorial Lecture 2008Angela Vincent
Neurosciences Group, Department of Clinical Neurology, John Radcliffe Hospital, University of Oxford, OX3 9TH
J Neuroimmunol 201:245-9. 2008..This review will highlight some of his most seminal findings and those that are following on from his work...
Diseases of the neuromuscular junctionJohn McConville
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DS, UK
Curr Opin Pharmacol 2:296-301. 2002..This is proving relevant to seronegative myasthenia gravis, with the discovery of anti-MuSK antibodies, and to a type of congenital myasthenic syndrome, in which acetylcholine receptor deficiency is due to mutations in rapsyn...
Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titersJacqueline Palace
DM, Department of Clinical Neurology, John Radcliffe Hospital, University of Oxford, Level 3 West Wing, Oxford OX3 9DU, England
Arch Neurol 67:1016-7. 2010..To describe a patient with neuromyelitis optica (NMO) whose aquaporin 4 (AQP4) antibody levels increased following treatment with interferon beta...
Autoantibodies associated with diseases of the CNS: new developments and future challengesAngela Vincent
Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK
Lancet Neurol 10:759-72. 2011....
Autoimmunity to ion-channels and other proteins in paraneoplastic disordersB Lang
Institute of Molecular Medicine, Oxford University, John Radclife Hospital, Headington, UK
Curr Opin Immunol 8:865-71. 1996..In the past year, there have been advances in the definition of some of the antigens that are recognized by patients' antibodies, and new observations on the results of passive and active immunization against the antigens...
Induction of primary immune responses by allogeneic human myoblasts: dissection of the cell types required for proliferation, IFNgamma secretion and cytotoxicityS J Curnow
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
J Neuroimmunol 86:53-62. 1998..These results suggest that human myoblasts can act as antigen-presenting cells for naive T lymphocytes, but only with additional co-stimulation...
Oxaliplatin induces hyperexcitability at motor and autonomic neuromuscular junctions through effects on voltage-gated sodium channelsRichard G Webster
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, UK
Br J Pharmacol 146:1027-39. 2005..The similarity between beta-pompilidotoxin and oxaliplatin suggests that alteration of voltage-gated Na(+) channel kinetics is likely to underlie the acute neurotoxic actions of oxaliplatin...
Autoimmune channelopathiesCamilla Buckley
University of Oxford, UK
Nat Clin Pract Neurol 1:22-33. 2005....
Presynaptic neuronal antigens expressed by a small cell lung carcinoma cell lineM Benatar
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford OX3 9DS, UK
J Neuroimmunol 113:153-62. 2001..These results suggest that many potential antigenic targets are present in SCLC, and indicate a surprising 'motor nerve terminal'-like characteristic of this line...
Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravisC Buckley
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom
Ann Neurol 50:64-72. 2001..The results also provide a new approach for early diagnosis of thymoma recurrence...
AChR phosphorylation and indirect inhibition of AChR function in seronegative MGC P Plested
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, University of Oxford, UK
Neurology 59:1682-8. 2002..Identifying the target for this factor should lead to improved diagnosis of MG in MuSK antibody-negative patients and may provide new insights into the function of the neuromuscular junction and pathophysiological mechanisms in MG...
Mutations in congenital myasthenic syndromes reveal an epsilon subunit C-terminal cysteine, C470, crucial for maturation and surface expression of adult AChRJohn Ealing
Neurosciences Group, Weatherall Institute of Molecular Medicine, The John Radcliffe, Oxford OX3 9DS, UK
Hum Mol Genet 11:3087-96. 2002....
Voltage-gated potassium channel antibodies in limbic encephalitisPatricia Pozo-Rosich
Neurosciences Group, Weatherall Insitute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, United Kingdom
Ann Neurol 54:530-3. 2003..VGKC antibodies do not unambiguously discriminate between idiopathic or paraneoplastic LE but probably indicate a good response to immunotherapy...
Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient miceA E Deconinck
Department of Biochemistry, University of Oxford, United Kingdom
J Cell Biol 136:883-94. 1997..Defects of utrophin could underlie some forms of congenital myasthenic syndrome in which a reduction of postsynaptic folds is observed...
Acetylcholine receptor delta subunit mutations underlie a fast-channel myasthenic syndrome and arthrogryposis multiplex congenitaS Brownlow
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford, United Kingdom
J Clin Invest 108:125-30. 2001..This is the first report of the association of AChR gene mutations with arthrogryposis multiplex congenita. It is probable that mutations that severely disrupt function of fetal AChR will underlie additional cases...
Autoimmunity in paraneoplastic neurological disordersA Vincent
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Headington, UK
J Neuroimmunol 84:105-9. 1998..This conference and workshop was an opportunity to discuss the clinical associations of these antibodies, the relevance of new antibody specificities, and to establish protocols for their detection...
Mutations in different functional domains of the human muscle acetylcholine receptor alpha subunit in patients with the slow-channel congenital myasthenic syndromeR Croxen
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
Hum Mol Genet 6:767-74. 1997....
Revised diagnostic criteria for neuromyelitis optica--incorporation of NMO-IgG statusSven Jarius
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DS, UK
Nat Clin Pract Neurol 3:E1. 2007
Low-voltage-activated A-current controls the firing dynamics of mouse hypothalamic orexin neuronsDenis Burdakov
Laboratory of Physiology, University of Oxford, Oxford, United Kingdom
Eur J Neurosci 20:3281-5. 2004..These results identify an important determinant of the firing dynamics of orexin neurons, and support the idea that the A-current can control neuronal gain...
A mouse model of AChR deficiency syndrome with a phenotype reflecting the human conditionJudy Cossins
Neuroscience Group, Weatherall Institute of Molecular Medicine, The John Radcliffe, Oxford, UK
Hum Mol Genet 13:2947-57. 2004....
Myasthenia gravis: diagnostic and management dilemmasJ Palace
University Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK
Curr Opin Neurol 14:583-9. 2001..We review the important issue of how treatments are assessed, and examine future treatment trial designs...
Absence of antibodies to glutamate receptor type 3 (GluR3) in Rasmussen encephalitisR Watson
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, UK
Neurology 63:43-50. 2004..CONCLUSIONS: GluR3 antibodies were only infrequently found in Rasmussen encephalitis or intractable epilepsy...
Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis opticaS Viegas
Department of Clinical Neurology, John Radcliffe Hospital, Oxford, UK
J Neurol Neurosurg Psychiatry 80:679-82. 2009..She died 21 months after presentation. Postmortem examination was highly suggestive of neuromyelitis optica, which was subsequently confirmed following the identification of aquaporin 4 antibodies...
Hypothermia in VGKC antibody-associated limbic encephalitisS Jacob
Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK
J Neurol Neurosurg Psychiatry 79:202-4. 2008..Both of these novel symptoms responded well to immunosuppressive therapy, with concurrent amelioration of amnesia/seizures...
Mutation of the acetylcholine receptor epsilon-subunit promoter in congenital myasthenic syndromeP Nichols
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford, UK
Ann Neurol 45:439-43. 1999....
Serum antibodies in epilepsy and seizure-associated disordersK McKnight
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DS, United Kingdom
Neurology 65:1730-6. 2005..Further studies are needed to determine whether the antibodies are pathogenic...
