Michael J Strong

Summary

Affiliation: University of Western Ontario
Country: Canada

Publications

  1. pmc Analysis of novel NEFL mRNA targeting microRNAs in amyotrophic lateral sclerosis
    Muhammad Ishtiaq
    Molecular Medicine Research Group, Robarts Research Institute, Western University, London, Ontario, Canada
    PLoS ONE 9:e85653. 2014
  2. doi The frontotemporal syndromes of ALS. Clinicopathological correlates
    Michael Joseph Strong
    Robarts Research Institute, Schulich School of Medicine and Dentistry, The University of Western Ontario, London, ON, Canada
    J Mol Neurosci 45:648-55. 2011
  3. doi TDP-43 and FUS/TLS: sending a complex message about messenger RNA in amyotrophic lateral sclerosis?
    Michael J Strong
    Department of Clinical Neurological Sciences and Robarts Research Institute, Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada
    FEBS J 278:3569-77. 2011
  4. doi Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury
    Katie Moisse
    Molecular Brain Research Group, Robarts Research Institute, PO Box 5015, 100 Perth Drive, London ON, Canada
    Brain Res 1296:176-86. 2009
  5. doi Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)
    Kathryn Volkening
    Molecular Brain Research Group, Robarts Research Institute, 100 Perth Drive, London, Ontario, Canada N6A 5K8
    Brain Res 1305:168-82. 2009
  6. doi Rho guanine nucleotide exchange factor is an NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis
    Cristian A Droppelmann
    Molecular Brain Research Group, Robarts Research Institute, University of Western Ontario, London, Ontario, Canada
    Neurobiol Aging 34:248-62. 2013
  7. doi Tau phosphorylation at threonine-175 leads to fibril formation and enhanced cell death: implications for amyotrophic lateral sclerosis with cognitive impairment
    May Gohar
    The Robarts Research Institute, The University of Western Ontario, London, Ontario, Canada
    J Neurochem 108:634-43. 2009
  8. doi Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism
    Brian A Keller
    Department of Pathology, Western University, London, ON, Canada
    Acta Neuropathol 124:733-47. 2012
  9. doi Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury
    Katie Moisse
    Molecular Brain Research Group, Robarts Research Institute, PO Box 5015, 100 Perth Drive, London ON, Canada N6A 5K8
    Brain Res 1249:202-11. 2009
  10. doi The complement factor C5a receptor is upregulated in NFL-/- mouse motor neurons
    Saima Humayun
    Department of Pathology, Schulich School of Medicine, University of Western Ontario, London, Canada
    J Neuroimmunol 210:52-62. 2009

Collaborators

Detail Information

Publications49

  1. pmc Analysis of novel NEFL mRNA targeting microRNAs in amyotrophic lateral sclerosis
    Muhammad Ishtiaq
    Molecular Medicine Research Group, Robarts Research Institute, Western University, London, Ontario, Canada
    PLoS ONE 9:e85653. 2014
    ....
  2. doi The frontotemporal syndromes of ALS. Clinicopathological correlates
    Michael Joseph Strong
    Robarts Research Institute, Schulich School of Medicine and Dentistry, The University of Western Ontario, London, ON, Canada
    J Mol Neurosci 45:648-55. 2011
    ....
  3. doi TDP-43 and FUS/TLS: sending a complex message about messenger RNA in amyotrophic lateral sclerosis?
    Michael J Strong
    Department of Clinical Neurological Sciences and Robarts Research Institute, Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada
    FEBS J 278:3569-77. 2011
    ..Coupled with the observation that mutations in either TDP-43 or FUS/TLS are associated with ALS, this provides further support for the integral role of altered RNA metabolism in ALS...
  4. doi Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury
    Katie Moisse
    Molecular Brain Research Group, Robarts Research Institute, PO Box 5015, 100 Perth Drive, London ON, Canada
    Brain Res 1296:176-86. 2009
    ..These results further support that TDP-43 is involved in neurofilament mRNA metabolism and transport, and provide insight into the pathogenesis of motor neuron death in ALS in which NFL mRNA levels are selectively suppressed...
  5. doi Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)
    Kathryn Volkening
    Molecular Brain Research Group, Robarts Research Institute, 100 Perth Drive, London, Ontario, Canada N6A 5K8
    Brain Res 1305:168-82. 2009
    ..These data suggest that NFL mRNA processing is fundamentally altered in ALS spinal motor neurons to favour compartmentalization within both stress granules and P-bodies, and that TDP-43 plays a fundamental role in this process...
  6. doi Rho guanine nucleotide exchange factor is an NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis
    Cristian A Droppelmann
    Molecular Brain Research Group, Robarts Research Institute, University of Western Ontario, London, Ontario, Canada
    Neurobiol Aging 34:248-62. 2013
    ..This is also the first described link between ALS and an RNA binding protein with aggregate formation that is also a central cell signaling pathway molecule...
  7. doi Tau phosphorylation at threonine-175 leads to fibril formation and enhanced cell death: implications for amyotrophic lateral sclerosis with cognitive impairment
    May Gohar
    The Robarts Research Institute, The University of Western Ontario, London, Ontario, Canada
    J Neurochem 108:634-43. 2009
    ..Both tau fibril formation and cell death were significantly enhanced in the presence of Thr175-Asp-tau, regardless of the tau isoform, suggesting that phosphorylation of Thr175 is associated with tau fibril formation in ALSci...
  8. doi Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism
    Brian A Keller
    Department of Pathology, Western University, London, ON, Canada
    Acta Neuropathol 124:733-47. 2012
    ..We also conclude that routine IHC-based analysis of spinal MNs may aid in the identification of families not previously suspected to harbor SOD1 mutations...
  9. doi Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury
    Katie Moisse
    Molecular Brain Research Group, Robarts Research Institute, PO Box 5015, 100 Perth Drive, London ON, Canada N6A 5K8
    Brain Res 1249:202-11. 2009
    ..These findings suggest that the upregulation of TDP-43 expression with prominent cytosolic localization in motor neurons injured by degenerative processes such as ALS may actually represent an appropriate response to neuronal injury...
  10. doi The complement factor C5a receptor is upregulated in NFL-/- mouse motor neurons
    Saima Humayun
    Department of Pathology, Schulich School of Medicine, University of Western Ontario, London, Canada
    J Neuroimmunol 210:52-62. 2009
    ..C5aR expression was increased in motor neurons in ALS. This data suggests that the early upregulation of C5aR may contribute to motor neuron damage that potentiates excitotoxicity in ALS...
  11. ncbi Widespread cerebral haemodynamics disturbances occur early in amyotrophic lateral sclerosis
    Matthew J Murphy
    Imaging Program, Lawson Health Research Institute, London, Ontario, Canada
    Amyotroph Lateral Scler 13:202-9. 2012
    ..Our findings suggest early widespread changes in CP occur outside the motor area in the absence of CI in ALS...
  12. ncbi Loss of nitric oxide-mediated down-regulation of NMDA receptors in neurofilament aggregate-bearing motor neurons in vitro: implications for motor neuron disease
    Teresa 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...
  13. ncbi 14-3-3 protein binds to the low molecular weight neurofilament (NFL) mRNA 3' UTR
    Wei 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...
  14. ncbi Neuronal tissue-specific ribonucleoprotein complex formation on SOD1 mRNA: alterations by ALS SOD1 mutations
    Wei 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...
  15. ncbi Temporal profiles of neuronal degeneration, glial proliferation, and cell death in hNFL(+/+) and NFL(-/-) mice
    Jesse R McLean
    Department of Pathology, Schulich School of Medicine, University of Western Ontario, London, Ontario, Canada
    Glia 52:59-69. 2005
    ....
  16. ncbi Calcium mediated excitotoxicity in neurofilament aggregate-bearing neurons in vitro is NMDA receptor dependant
    Teresa 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...
  17. pmc Altered microRNA expression profile in Amyotrophic Lateral Sclerosis: a role in the regulation of NFL mRNA levels
    Danae Campos-Melo
    Molecular Brain Research Group, Robarts Research Institute, University of Western Ontario, London, ON, Canada
    Mol Brain 6:26. 2013
    ..Furthermore, we performed functional analysis to identify a group of dysregulated miRNAs that could be responsible for the selective suppression of low molecular weight neurofilament (NFL) mRNA observed in ALS...
  18. ncbi TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein
    Michael 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...
  19. ncbi Innate immunity in amyotrophic lateral sclerosis
    Katie 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
    ....
  20. doi Cerebral haemodynamic changes accompanying cognitive impairment in primary lateral sclerosis
    Matthew J Murphy
    Imaging Program, Lawson Health Research Institute, London, Ontario, Canada
    Amyotroph Lateral Scler 9:359-68. 2008
    ..These observations suggest that a subset of PLS patients is subject to cognitive decline and that this process is associated with changes in cerebral haemodynamics...
  21. pmc Transient middle cerebral artery occlusion induces microglial priming in the lumbar spinal cord: a novel model of neuroinflammation
    Katie 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...
  22. doi Detection of a novel frameshift mutation and regions with homozygosis within ARHGEF28 gene in familial amyotrophic lateral sclerosis
    Cristian A Droppelmann
    Molecular Brain Research Group, Robarts Research Institute, University of Western Ontario, London, Ontario
    Amyotroph Lateral Scler Frontotemporal Degener 14:444-51. 2013
    ..In conclusion, our findings of genetic alterations in the ARHGEF28 gene in cases of FALS suggest that a more comprehensive genetic analysis would be warranted...
  23. doi 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...
  24. doi Human low molecular weight neurofilament (NFL) mRNA interacts with a predicted p190RhoGEF homologue (RGNEF) in humans
    Kathryn Volkening
    Robarts Research Institute, London, Ontario, Canada
    Amyotroph Lateral Scler 11:97-103. 2010
    ....
  25. ncbi Widespread neuronal and glial hyperphosphorylated tau deposition in ALS with cognitive impairment
    Wencheng Yang
    Robarts Research Institute, The University of Western Ontario, Canada
    Amyotroph Lateral Scler 13:178-93. 2012
    ..In conclusion, ALSci is unique in possessing both tau and TDP-43 pathology. The presence of widespread astrocytic tau pathology suggests that ALSci may initially be characterized by astrocytic pathology...
  26. ncbi Microtubule-associated tau protein positive neuronal and glial inclusions in ALS
    Wencheng Yang
    Robarts Research Institute, The University of Western Ontario, London, Canada
    Neurology 61:1766-73. 2003
    ....
  27. ncbi Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up
    Maria 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...
  28. ncbi Tau protein aggregation in the frontal and entorhinal cortices as a function of aging
    Wencheng 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...
  29. ncbi Activated microglial supernatant induced motor neuron cytotoxicity is associated with upregulation of the TNFR1 receptor
    Weiyan 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...
  30. ncbi 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...
  31. ncbi Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis
    Michael J Strong
    Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario, Canada
    Amyotroph Lateral Scler 10:131-46. 2009
    ....
  32. ncbi Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNA
    Wei 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...
  33. ncbi RNA metabolism in ALS: When normal processes become pathological
    Cristian A Droppelmann
    Molecular Medicine Group, Robarts Research Institute, Western University, London, Ontario
    Amyotroph Lateral Scler Frontotemporal Degener . 2014
    ....
  34. ncbi Intermediate filament steady-state mRNA levels in amyotrophic lateral sclerosis
    Michael 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...
  35. doi The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis
    Michael J Strong
    Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario, Canada
    Amyotroph Lateral Scler 9:323-38. 2008
    ....
  36. pmc Amyotrophic lateral sclerosis: update for family physicians
    Christen L Shoesmith
    London Health Sciences Centre, Ontario, Canada
    Can Fam Physician 52:1563-9. 2006
    ..To discuss the epidemiology, pathogenesis, diagnosis, expected course, prognosis, and treatment of amyotrophic lateral sclerosis (ALS), a degenerative disorder of the nervous system associated with progressive weakness...
  37. ncbi Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis
    Hsueh Ning Liu
    Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada
    Ann Neurol 66:75-80. 2009
    ..It has recently been proposed that misfolded SOD1 may be causative of all types of ALS, including sporadic cases (sALS). In the present study, we have used a specific antibody to test for the presence of monomer/misfolded SOD1 in sALS...
  38. pmc C9ORF72 repeat expansions in cases with previously identified pathogenic mutations
    Marka van Blitterswijk
    From the Departments of Neuroscience M v B, M C B, M D H, M E M, N J R, P E B, T R, B M, P E A A, K F B, L P, D W D, R R and Neurology Z K W, K B B, N R G R, Mayo Clinic, Jacksonville, FL Proteomics Unit and NeuroBioGen Lab Memory Clinic R G, L B, G B, IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy Department of Clinical Neurological Sciences E F, M J S, Schulich School of Medicine and Dentistry, The University of Western Ontario, London, Canada Division of Neurology G Y R H, and Department of Pathology and Laboratory Medicine I R M, University of British Columbia, Vancouver, Canada Department of Neurology B J K, D S K, R C P, Healthcare Hawkes Bay
    Neurology 81:1332-41. 2013
    ....
  39. doi Longitudinal changes in discourse production in amyotrophic lateral sclerosis
    Angela Roberts-South
    School of Communication Sciences and Disorders, University of Western Ontario, London, Ontario, Canada
    Semin Speech Lang 33:79-94. 2012
    ..The results suggest that subtle cognitive language deficits that affect discourse emerge early in ALS and progress with disease progression...
  40. ncbi Sequestration of nNOS in neurofilamentous aggregate bearing neurons in vitro leads to enhanced NMDA-mediated calcium influx
    Teresa R Sanelli
    Robarts Research Institute and Department of Pathology, The University of Western Ontario, London, Ontario, Canada
    Brain Res 1004:8-17. 2004
    ....
  41. ncbi Amyotrophic lateral sclerosis: contemporary concepts in etiopathogenesis and pharmacotherapy
    Michael 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...
  42. ncbi 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...
  43. doi 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...
  44. ncbi Cognitive impairment, frontotemporal dementia, and the motor neuron diseases
    Michael J Strong
    Department of Clinical Neurological Sciences and the Robarts Research Institute, London, Ontario, Canada
    Ann Neurol 54:S20-3. 2003
  45. ncbi NIPPV: prevalence, approach and barriers to use at Canadian ALS centres
    Benjamin R Ritsma
    Schulich School of Medicine and Dentistry, The University of Western Ontario, London, Ontario, Canada
    Can J Neurol Sci 37:54-60. 2010
    ....
  46. ncbi Increasing peak expiratory flow time in amyotrophic lateral sclerosis
    Steven R Wilson
    Division of Respirology, London Health Sciences Center, London, ON, Canada
    Chest 127:156-60. 2005
    ..Increasing peak expiratory flow time (PEFT) may represent a further sensitive measure of declining respiratory muscle strength in ALS...
  47. ncbi Selective loss of trans-acting instability determinants of neurofilament mRNA in amyotrophic lateral sclerosis spinal cord
    Wei 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...
  48. pmc Prognosis of amyotrophic lateral sclerosis with respiratory onset
    Christen 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...
  49. ncbi The basic aspects of therapeutics in amyotrophic lateral sclerosis
    Michael 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...