Research Topics
Genomes and GenesSpecies | I R A MackenzieSummaryAffiliation: University of British Columbia Country: Canada Publications
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Publications
The relationship between extramotor ubiquitin-immunoreactive neuronal inclusions and dementia in motor neuron diseaseIan R A Mackenzie
Department of Pathology, University of British Columbia, Vancouver General Hospital, 855 West 12th Avenue, Canada V5Z 1M9
Acta Neuropathol 105:98-102. 2003..Such cases may either represent a subclinical stage of pathology or indicate that cognitive dysfunction is an underrecognized complication of MND...- The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin geneIan R A Mackenzie
Department of Pathology and Laboratory Medicine, University of British Columbia and Vancouver Coastal Health Vancouver, BC, Canada
Brain 129:3081-90. 2006..These findings suggest that FTD caused by PGRN mutations has a recognizable pathology with the most characteristic feature being ub-ir NII... - Manson's schistosomiasis presenting as a brain tumor. Case reportI R Mackenzie
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
J Neurosurg 89:1052-4. 1998..This case demonstrates that neuroschistosomiasis should be considered when an individual presenting with an intracerebral mass has lived in a region in which this disease is endemic... - Anti-inflammatory drugs and Alzheimer-type pathology in agingI R Mackenzie
Department of Pathology and Laboratory Medicine, Vancouver General Hospital and the University of British Columbia, Canada
Neurology 54:732-4. 2000.... - Postmortem studies of the effect of anti-inflammatory drugs on Alzheimer-type pathology and associated inflammationI R Mackenzie
Department of Pathology and Laboratory Medicine, Vancouver General Hospital and the University of British Columbia, Vancouver, BC, Canada
Neurobiol Aging 22:819-22. 2001..The results of these and future postmortem studies will be invaluable in the development of optimum treatment strategies... - Neuronal intranuclear inclusions distinguish familial FTD-MND type from sporadic casesIan R A Mackenzie
Department of Pathology, Vancouver General Hospital, 855 West 12th Avenue, V5Z 1M9, Vancouver, British Columbia, Canada
Acta Neuropathol 105:543-8. 2003.... - Extrapyramidal features in patients with motor neuron disease and dementia; a clinicopathological correlative studyIan R Mackenzie
Department of Pathology, Vancouver General Hospital and University of British Columbia, 855 West 12th Avenue, V5Z 1M9, Vancouver, British Columbia, Canada
Acta Neuropathol 107:336-40. 2004..This study illustrates the utility of ubiquitin immunohistochemistry in demonstrating the range of pathology in MND and provides a neuropathological correlate for the extrapyramidal features which may occur in MND with dementia... - Neurofilament inclusion body disease with early onset frontotemporal dementia and primary lateral sclerosisI R A Mackenzie
Division of Neuropathology, Department of Pathology, University of British Columbia, Vancouver, Canada
Clin Neuropathol 23:183-93. 2004..In addition, it extends the clinical phenotype of NIBD to include PLS and better defines the anatomical distribution and morphology of the pathological lesions... - Neuronal intranuclear inclusions distinguish familial FTD-MND type from sporadic casesIan R A Mackenzie
Department of Pathology, University of British Columbia, Vancouver, Canada
Dement Geriatr Cogn Disord 17:333-6. 2004.... 
Progranulin expression in the developing and adult murine brainTerri L Petkau
Centre for Molecular Medicine and Therapeutics, Department of Medical Genetics, University of British Columbia, and Children s and Women s Hospital, Vancouver, BC, Canada V5Z 4H4
J Comp Neurol 518:3931-47. 2010..Our results suggest that progranulin plays distinct roles in neurons and microglia, both of which likely contribute to overall neuronal health and function...- The molecular genetics and neuropathology of frontotemporal lobar degeneration: recent developmentsIan R A Mackenzie
Department of Pathology, Vancouver General Hospital, 855 West 12th Avenue, Vancouver, BC, V5Z 1M9, Canada
Neurogenetics 8:237-48. 2007..It is anticipated that these discoveries will facilitate the development of new diagnostic tests and therapeutics... 
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusionsNigel J Cairns
MRCPath, Department of Pathology and Immunology, Washington University School of Medicine, Campus Box 8118, St Louis, MO 63110, USA
Am J Pathol 171:227-40. 2007....- Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar DegenerationNigel J Cairns
Department of Neurology, Washington University School of Medicine, Campus Box 8118, 660 South Euclid Avenue, St Louis, MO, 63110, USA
Acta Neuropathol 114:5-22. 2007..These criteria will be of value to the practicing neuropathologist and provide a foundation for clinical, clinico-pathologic, mechanistic studies and in vivo models of pathogenesis of FTLD... - Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusionsIan R A Mackenzie
Department of Pathology, University of British Columbia, Vancouver General Hospital, 855 West 12th Avenue, Vancouver, British Columbia, V5Z 1M9 Canada
Brain 131:1282-93. 2008..Moreover, the existence of such cases indicates that the designations of 'FTLD-U' and 'TDP-43 proteinopathy' should not be considered to be synonymous... - Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43Yvonne Davidson
Clinical Neuroscience Research Group, Division of Medicine and Neuroscience, Greater Manchester Neurosciences Centre, Hope Hospital, University of Manchester, Salford, M6 8HD, UK
Acta Neuropathol 113:521-33. 2007..We conclude that the UBQ-ir lesions of FTLD and MND are defined by the presence of TDP-43, and that these disorders can be subsumed into a single disease entity under the umbrella of TDP-43 proteinopathy... - Mutations in progranulin explain atypical phenotypes with variants in MAPTStuart M Pickering-Brown
Brain 129:3124-6. 2006..Here, we demonstrate that the MAPT variants are almost certainly rare benign polymorphisms as all of these cases harbour mutations in Progranulin (PGRN). Mutations in PGRN were recently shown to cause ubiquitin-positive FTDP-17... - The neuropathology and biochemistry of frontotemporal dementiaDavid G Munoz
Department of Pathology, University of Western Ontario, London, Ontario, Canada
Ann Neurol 54:S24-8. 2003 - Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotypeIan R A Mackenzie
Department of Pathology, Vancouver General Hospital, V5Z 1M9, Vancouver, BC, Canada
Acta Neuropathol 112:539-49. 2006.... - Dementia lacking distinctive histology (DLDH) revisitedIan R A Mackenzie
Department of Pathology, Vancouver General Hospital, Vancouver, BC, Canada
Acta Neuropathol (Berl) 112:551-9. 2006..Hence, we conclude that DLDH is a very rare disorder, and that lack of sensitivity for UBQ immunostaining is likely responsible for the failure to disclose this pathology and to provide a diagnosis of FTLD-U... - Ubiquitin immunohistochemistry suggests classic motor neuron disease, motor neuron disease with dementia, and frontotemporal dementia of the motor neuron disease type represent a clinicopathologic spectrumIan R A Mackenzie
Division of Neuropathology, University of British Columbia, Vancouver, Canada
J Neuropathol Exp Neurol 64:730-9. 2005..The only finding restricted to a specific subgroup of patients was the presence of ub-ir neuronal intranuclear inclusions in some cases of familial FTD... - TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLDSigrun Roeber
Center for Neuropathology and Prion Research, Ludwig Maximilians University Munich, Feodor Lynen Str 23, 81377, Munich, Germany
Acta Neuropathol 116:147-57. 2008..The highly consistent clinical and neuropathological phenotype supports the concept that TDP-43-negative FTLD-U should be considered as a new clinicopathological FTLD entity... - A reassessment of the neuropathology of frontotemporal dementia linked to chromosome 3Ida Elisabeth Holm
Department of Pathology, Aalborg Hospital, Aarhus University Hospital, Aalborg, Denmark
J Neuropathol Exp Neurol 66:884-91. 2007.... - Clinical presentation of prodromal frontotemporal dementiaBradley J Hallam
Division of Neurology, Geriatric Psychiatry Outreach Team, Vancouver Hospital, Vancouver, British Columbia
Am J Alzheimers Dis Other Demen 22:456-67. 2007.... - The neuropathology of FTD associated With ALSIan R A Mackenzie
Department of Pathology, University of British Columbia and Vancouver General Hospital, British Columbia, Canada
Alzheimer Dis Assoc Disord 21:S44-9. 2007..Together, these findings suggest that FTD-ALS is part of a clinicopathologic spectrum of disease, now identified as TDP-43 proteinopathies... - Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutationsIan R A Mackenzie
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Ann Neurol 61:427-34. 2007..We recently identified TDP-43 as the major pathological protein in sporadic ALS. In this study, we investigated TDP-43 in a larger series of ALS cases (n = 111), including familial cases with and without SOD1 mutations... - The neuropathology and clinical phenotype of FTD with progranulin mutationsIan R A Mackenzie
Department of Pathology, Vancouver General Hospital, 855 West 12th Avenue, Vancouver, BC, Canada
Acta Neuropathol 114:49-54. 2007..This degree of clinical variability makes it difficult to predict which cases of familial FTD will turn out to have a PGRN mutation... - Progranulin in frontotemporal lobar degeneration and neuroinflammationZeshan Ahmed
Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, USA
J Neuroinflammation 4:7. 2007.... - alpha-internexin is present in the pathological inclusions of neuronal intermediate filament inclusion diseaseNigel J Cairns
Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4283, USA
Am J Pathol 164:2153-61. 2004..The discovery of alpha-internexin in the cytoplasmic inclusions implicates novel mechanisms of pathogenesis in NIFID and other neurological diseases with pathological accumulations of IFs... - alpha-Internexin aggregates are abundant in neuronal intermediate filament inclusion disease (NIFID) but rare in other neurodegenerative diseasesNigel J Cairns
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 3600 Spruce Street, Philadelphia, PA 19104 4283, USA
Acta Neuropathol 108:213-23. 2004..The discovery of alpha-internexin in neuronal cytoplasmic inclusions implicates novel mechanisms of pathogenesis in NIFID and other neurological diseases with pathological filamentous neuronal inclusions...