Ian R. A. Mackenzie
University of British Columbia
272 Papers
1K Citations
Ian R. A. Mackenzie is an academic researcher from University of British Columbia. The author has contributed to research in topics: Frontotemporal dementia & Frontotemporal lobar degeneration. The author has an hindex of 89, co-authored 240 publications. Previous affiliations of Ian R. A. Mackenzie include Vancouver General Hospital.
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Papers
A harmonized classification system for FTLD-TDP pathology
Ian R. A. Mackenzie,Manuela Neumann,Atik Baborie,Deepak M. Sampathu,Daniel du Plessis,Evelyn Jaros,Robert H. Perry,John Q. Trojanowski,David M. A. Mann,Virginia M.-Y. Lee +9 more
TL;DR: In this article, Sampathu et al. proposed a new classification system for FTLD-TDP pathology, which is a single harmonized system that replaces the two currently in use.
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
Dorothee Dormann,Dorothee Dormann,Ramona Rodde,Ramona Rodde,Dieter Edbauer,Eva Bentmann,Eva Bentmann,Ingeborg Fischer,Alexander Hruscha,Manuel E Than,Ian R. A. Mackenzie,Anja Capell,Anja Capell,Bettina Schmid,Manuela Neumann,Christian Haass,Christian Haass +16 more
TL;DR: It is proposed that two pathological hits, namely nuclear import defects and cellular stress, are involved in the pathogenesis of FUS‐opathies.
A new subtype of frontotemporal lobar degeneration with FUS pathology.
Manuela Neumann,Rosa Rademakers,Sigrun Roeber,Matt Baker,Hans A. Kretzschmar,Ian R. A. Mackenzie +5 more
TL;DR: Findings suggest that FUS is the pathological protein in a significant subgroup of sporadic FTD and reinforce the concept that FTd and amyotrophic lateral sclerosis are closely related conditions.
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Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation.
Hansen Lui,Jiasheng Zhang,Stefanie Ritter Makinson,Michelle K. Cahill,Kevin W. Kelley,Hsin-Yi Huang,Yulei Shang,Michael C. Oldham,Lauren Herl Martens,Fuying Gao,Giovanni Coppola,Steven A. Sloan,Christine L. Hsieh,Charles C. Kim,Eileen H. Bigio,Sandra Weintraub,M.-Marsel Mesulam,Rosa Rademakers,Ian R. A. Mackenzie,William W. Seeley,Anna Karydas,Bruce L. Miller,Barbara Borroni,Roberta Ghidoni,Robert V. Farese,Jeanne T. Paz,Ben A. Barres,Eric J. Huang,Eric J. Huang +28 more
TL;DR: In this paper, the authors used transcriptome profiling to demonstrate that deficiency in frontotemporal dementia (FTD) gene progranulin (Grn) leads to an age-dependent, progressive upregulation of lysosomal and innate immunity genes, increased complement production, and enhanced synaptic pruning in microglia.
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TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics
Ian R. A. Mackenzie,Alexandra M. Nicholson,Mohona Sarkar,James Messing,Maria D. Purice,Cyril Pottier,Kavya Annu,Matt Baker,Ralph B. Perkerson,Aishe Kurti,Billie J. Matchett,Tanja Mittag,Jamshid Temirov,Ging-Yuek Robin Hsiung,Charles Krieger,Charles Krieger,Melissa E. Murray,Masato Kato,John D. Fryer,Leonard Petrucelli,Lorne Zinman,Sandra Weintraub,M.-Marsel Mesulam,Julia Keith,Sasha Zivkovic,Veronica Hirsch-Reinshagen,Raymond P. Roos,Stephan Züchner,Neill R. Graff-Radford,Ronald C. Petersen,Richard J. Caselli,Zbigniew K. Wszolek,Elizabeth Finger,Carol F. Lippa,Carol F. Lippa,David Lacomis,Heather Stewart,Dennis W. Dickson,Hong Joo Kim,Ekaterina Rogaeva,Eileen H. Bigio,Kevin B. Boylan,J. Paul Taylor,Rosa Rademakers +43 more
TL;DR: The identification of TIA1 mutations in ALS/FTD reinforces the importance of RNA metabolism and SG dynamics in ALS-FTD pathogenesis.
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