Research Topics
Species | Michael StrongSummaryAffiliation: University of Western Ontario Country: Canada Publications
| Collaborators
|
Detail Information
Publications
Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosisMichael J Strong
Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario, Canada
Amyotroph Lateral Scler 10:131-46. 2009....
Transient middle cerebral artery occlusion induces microglial priming in the lumbar spinal cord: a novel model of neuroinflammationKatie Moisse
Cell Biology Research Group, Robarts Research Institute, London, Ontario, Canada
J Neuroinflammation 5:29. 2008..Following permanent occlusion, neuropathological changes - including a robust glial inflammatory response - also occur downstream of the infarct in the spinal cord...- The basic aspects of therapeutics in amyotrophic lateral sclerosisMichael J Strong
Department of Clinical Neurological Sciences, Robarts Research Institute, Room 7OF 10, University Campus, London Health Sciences Centre, University of Western Ontario, 339 Windermere Road, London, Ontario, Canada N6A 5A5
Pharmacol Ther 98:379-414. 2003..Designing pharmacological strategies to mitigate against this process thus becomes an increasingly complex issue, which is reviewed in this article... - The pathobiology of amyotrophic lateral sclerosis: a proteinopathy?Michael J Strong
Robarts Research Institute, Department of Clinical Neurological Sciences, The University of Western Ontario, London, Canada
J Neuropathol Exp Neurol 64:649-64. 2005..The role of nonneuronal cells in mediating motor neuronal injury is discussed in the context of alterations in tyrosine kinase activity and enhanced protein phosphorylation... - Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis: discrete entities or spectrum?Michael J Strong
Department of Clinical Neurological Sciences, The University of Western Ontario, The Cell Biology Research Group, Roberts Research Institute, London, Ontario, Canada
Amyotroph Lateral Scler Other Motor Neuron Disord 6:8-16. 2005..Only through the development and application of exacting clinical diagnostic criteria to epidemiological studies, along with greater numbers of post-mortem examinations, however, will these questions be answered fully... - Amyotrophic lateral sclerosis: contemporary concepts in etiopathogenesis and pharmacotherapyMichael J Strong
Department of Clinical Neurological Sciences, University of Western Ontario, The Robarts Research Institute, London, Canada
Expert Opin Investig Drugs 13:1593-614. 2004..This review will highlight recent epidemiological and neurochemical advances in our understanding of ALS, and place them into the context of understanding the development of novel treatment avenues for this devastating disease... 
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)Michael J Strong
Molecular Brain Research Group, Robarts Research Institute, London, Ontario, Canada
J Neurol Sci 288:1-12. 2010..These observations lead directly to the hypothesis that ALS can be viewed as a disorder of RNA metabolism, thus providing a novel pathway for the development of molecular pharmacotherapies...- TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding proteinMichael J Strong
Cell Biology Research Group, Robarts Research Institute, London, Ontario, Canada
Mol Cell Neurosci 35:320-7. 2007..TDP43 is a unique hNFL mRNA-binding protein that is altered in its somatotopic localization in ALS spinal motor neurons and potentially contributes to the formation of NF aggregates in ALS through alterations in NF stoichiometry... - Intermediate filament steady-state mRNA levels in amyotrophic lateral sclerosisMichael J Strong
Cell Biology Research Group, Robarts Research Institute, London, Ont, Canada
Biochem Biophys Res Commun 316:317-22. 2004..These findings suggest that disturbances in both NFL and peripherin expression, independently known to contribute to the generation of motor neuron dysfunction in transgenic mice, are evident in ALS... - In vitro reactive nitrating species toxicity in dissociated spinal motor neurons from NFL (-/-) and hNFL (+/+) transgenic miceMichael Strong
Cell Biology Research Group, Robarts Research Institute, London, Ontario, Canada
Amyotroph Lateral Scler Other Motor Neuron Disord 4:81-9. 2003..These observations suggest that NFL deficient motor neurons are at an enhanced risk of cell death mediated by RNS... - Amyotrophic lateral sclerosis: a review of current conceptsMichael Strong
The Department of Clinical Neurological Sciences, The University of Western Ontario, London, Canada
Amyotroph Lateral Scler Other Motor Neuron Disord 4:136-43. 2003..Significant advances in both symptomatic and adjunctive therapy have resulted in prolonged quality and duration of life... - Tau protein hyperphosphorylation in sporadic ALS with cognitive impairmentM J Strong
Cell Biology Research Group, Robart s Research Institute, London, Ontario, Canada
Neurology 66:1770-1. 2006..Soluble tau from both AD and ALSci was also phosphorylated at S237. Tau hyperphosphorylation is associated with ALS... - NMDA induces NOS 1 translocation to the cell membrane in NGF-differentiated PC 12 cellsMark Arundine
The Department of Pathology, The University of Western Ontario, Rm 7 OF 10, UC LHSC, 339 Windermere Road, London, N6A 5C1, Ontario, Canada
Brain Res 976:149-58. 2003..Our data validate that NGF-differentiated PC12 cells may be employed as a useful in vitro model to further study the regulation of NOS1 subsequent to NMDAR activation... - The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosisMichael J Strong
Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario, Canada
Amyotroph Lateral Scler 9:323-38. 2008.... - Tau protein aggregation in the frontal and entorhinal cortices as a function of agingWencheng Yang
Robarts Research Institute, London, Ontario, Canada
Brain Res Dev Brain Res 156:127-38. 2005..In this paper we examined the age-dependant deposition of tau protein in the frontal and entorhinal neocortices... - Prognosis of amyotrophic lateral sclerosis with respiratory onsetChristen L Shoesmith
Department of Clinical Neurological Sciences, University of Western Ontario, London, Ontario, Canada
J Neurol Neurosurg Psychiatry 78:629-31. 2007..Non-invasive positive pressure ventilation (NIPPV) significantly improved survival compared with those who did not use NIPPV. This study suggests that ALS with respiratory onset does not necessarily follow a rapidly progressive course... - 14-3-3 protein binds to the low molecular weight neurofilament (NFL) mRNA 3' UTRWei Wen Ge
Cell Biology Research Group, Robarts Research Institute, 100 Perth Drive, London, Ontario, Canada N6A 5K8
Mol Cell Neurosci 34:80-7. 2007..These data show a novel interaction for 14-3-3 with NFL mRNA, and suggests that 14-3-3 may play a role in regulating NFL mRNA stability... - Calcium mediated excitotoxicity in neurofilament aggregate-bearing neurons in vitro is NMDA receptor dependantTeresa Sanelli
Department of Pathology, University of Western Ontario, London, ON, Canada
J Neurol Sci 256:39-51. 2007..These studies demonstrate that calcium mediated excitotoxicity in NF aggregate-bearing neurons is NMDA receptor dependant... - Loss of nitric oxide-mediated down-regulation of NMDA receptors in neurofilament aggregate-bearing motor neurons in vitro: implications for motor neuron diseaseTeresa Sanelli
Department of Pathology, University of Western Ontario and the Cell Biology Group, Robarts Research Institute, London, Ontario, Canada
Free Radic Biol Med 42:143-51. 2007..No effect was observed in aggregate-bearing neurons. These findings suggest that the presence of NF aggregates renders motor neurons more susceptible to NMDA-mediated excitotoxicity, and that this can be reversed by NO... - Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-upMaria Carmela Tartaglia
Clinical Neurological Sciences, London Health Sciences Centre, University of Western Ontario, 339 Windermere Road, London, Ontario, Canada
Arch Neurol 64:232-6. 2007..Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum... - Post-transcriptional control of neurofilaments in development and diseaseAmar Thyagarajan
Department of Biological Sciences and the Neuroscience Research Center, University at Albany, SUNY, 1400 Washington Avenue, Albany, NY 12222, USA
Exp Cell Res 313:2088-97. 2007.... - Upregulation of GSK3beta expression in frontal and temporal cortex in ALS with cognitive impairment (ALSci)Wencheng Yang
Robarts Research Institute, The University of Western Ontario, London, Ontario, Canada
Brain Res 1196:131-9. 2008..This supports the potential role for GSK3beta as a therapeutic target in ALS... - Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALSJanice Robertson
Centre for Research in Neurodegenerative Diseases, Department of Laboratory, Medicine and Pathobiology, University of Toronto, Toronto, Canada M5S 3H2
Neurosci Lett 420:128-32. 2007..Our findings indicate that the process of motor neuron degeneration in mutant SOD1 transgenic mice is unlikely to involve the abnormalities of TDP-43 described in the human disease... - Inhibition of Pin1 reduces glutamate-induced perikaryal accumulation of phosphorylated neurofilament-H in neuronsSashi Kesavapany
Cytoskeletal Protein Regulation Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 18:3645-55. 2007.... - Neuronal tissue-specific ribonucleoprotein complex formation on SOD1 mRNA: alterations by ALS SOD1 mutationsWei Wen Ge
Cell Biology Research Group, Robarts Research Institute, London, Ontario, Canada
Neurobiol Dis 23:342-50. 2006..This paper reports RNP complex formation on SOD1 mRNA as a neuronal tissue-specific and ALS-associated mutation sensitive feature... - Innate immunity in amyotrophic lateral sclerosisKatie Moisse
Cell Biology Research Group, Robarts Research Institute, Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario, Canada
Biochim Biophys Acta 1762:1083-93. 2006.... 
A neurotoxic peripherin splice variant in a mouse model of ALSJanice Robertson
Centre for Research in Neurosciences, Research Institute of the McGill University Health Centre, McGill University, Montreal, Quebec, H3G 1A4, Canada
J Cell Biol 160:939-49. 2003..This evidence suggests that expression of neurotoxic splice variants of peripherin may contribute to the neurodegenerative mechanism in ALS...- Selective loss of trans-acting instability determinants of neurofilament mRNA in amyotrophic lateral sclerosis spinal cordWei Wen Ge
Cell Biology Research Group, Robarts Research Institute, London, Ontario N6A 5K8, Canada
J Biol Chem 278:26558-63. 2003..These findings suggest that the trans-acting NFL-destabilizing elements are selectively suppressed in ALS homogenates, resulting in an increased stability and level of NFL mRNA... - Cognitive impairment, frontotemporal dementia, and the motor neuron diseasesMichael J Strong
Department of Clinical Neurological Sciences and the Robarts Research Institute, London, Ontario, Canada
Ann Neurol 54:S20-3. 2003 - High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1Zarah Batulan
Montreal Neurological Institute and Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada H3A 2B4
J Neurosci 23:5789-98. 2003..These results indicate that the high threshold for induction of the stress response in motor neurons stems from an impaired ability to activate the main heat shock-stress sensor, HSF1... - Microtubule-associated tau protein positive neuronal and glial inclusions in ALSWencheng Yang
Robarts Research Institute, The University of Western Ontario, London, Canada
Neurology 61:1766-73. 2003..Both gray and white matter soluble tau protein expression was similar among control, ALS, and ALSci cases. CONCLUSIONS: Cognitive dysfunction in ALS may reflect abnormal tau protein metabolism... - Activated p38MAPK is a novel component of the intracellular inclusions found in human amyotrophic lateral sclerosis and mutant SOD1 transgenic miceCaterina Bendotti
Laboratory of Molecular Neurobiology, Dept Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri, Via Eritrea, 62, 20157 Milano, Italy
J Neuropathol Exp Neurol 63:113-9. 2004..Our observations indicate that activation of p38MAPK might contribute significantly to the pathology of motor neurons in ALS... - Sequestration of nNOS in neurofilamentous aggregate bearing neurons in vitro leads to enhanced NMDA-mediated calcium influxTeresa R Sanelli
Robarts Research Institute and Department of Pathology, The University of Western Ontario, London, Ontario, Canada
Brain Res 1004:8-17. 2004.... - Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNAWei Wen Ge
Cell Biology Research Group, Robarts Research Institute, London, Ontario N6A 5K8, Canada
J Biol Chem 280:118-24. 2005..These observations provide an explanation for a novel gain of function in which mutant SOD1 expression in motor neurons alters an intermediate filament protein expression... - ALS--not what we thoughtMichael J Strong
Arch Neurol 63:319-20. 2006 - Activated microglial supernatant induced motor neuron cytotoxicity is associated with upregulation of the TNFR1 receptorWeiyan Wen
Cell Biology Research Group, Robarts Research Institute, London, Ont, Canada
Neurosci Res 55:87-95. 2006..The TNFR2 receptor was not involved. These observations have important implications in understanding the mechanism by which microglial activation contributes to the motor neuron degeneration... - Temporal profiles of neuronal degeneration, glial proliferation, and cell death in hNFL(+/+) and NFL(-/-) miceJesse R McLean
Department of Pathology, Schulich School of Medicine, University of Western Ontario, London, Ontario, Canada
Glia 52:59-69. 2005.... - Activated microglia (BV-2) facilitation of TNF-alpha-mediated motor neuron death in vitroBei Ping He
Neurodegeneration Research Group, The John P Robarts Research Institute, University of Western Ontario, London, Ontario, Canada N6A 5A5
J Neuroimmunol 128:31-8. 2002.... - TAU mutations are not a predominant cause of frontotemporal dementia in Canadian patientsAnastasia Levchenko
Center for Research in Neuroscience, McGill University Health Center Research Institute, Montreal, Quebec, Canada
Can J Neurol Sci 31:363-7. 2004..In this study, we evaluated the involvement of mutations in the TAU gene in development of frontotemporal dementia phenotype in patients of French or English Canadian origins... - An aggregate-inducing peripherin isoform generated through intron retention is upregulated in amyotrophic lateral sclerosis and associated with disease pathologyShangxi Xiao
Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada M5S 3H2
J Neurosci 28:1833-40. 2008..These findings are the first to establish that peripherin splicing abnormalities occur in ALS, generating aggregation-prone splice isoforms... - Amyotrophic lateral sclerosis: update for family physiciansChristen L Shoesmith
London Health Sciences Centre, Ontario, Canada
Can Fam Physician 52:1563-9. 2006..CONCLUSION: Because ALS is a complex disease, care of ALS patients is best provided at multidisciplinary clinics that specialize in managing patients with this disorder... - Increasing peak expiratory flow time in amyotrophic lateral sclerosisSteven R Wilson
Division of Respirology, London Health Sciences Center, London, ON, Canada
Chest 127:156-60. 2005..PEFT increases at a faster rate than the rate of decline in both FVC and PEFR. PEFT is a quantitative measure of decreasing pulmonary function in ALS that is easily measured... - Biochemical markers: summaryMichael J Strong
University of Western Ontario, Canada
Amyotroph Lateral Scler Other Motor Neuron Disord 3:S85-90. 2002