Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update
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Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration : an update
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Protein misfolding and aggregation is a cardinal feature of the post-mortem diagnostic criteria for frontotemporal lobar degeneration.
“In recent years, attention has focused on understanding the pathogenic role of protein misfolding and aggregation, which is a cardinal feature of the post-mortem diagnostic criteria for frontotemporal lobar degeneration (FTLD) (Mackenzie et al., 2010).”
From Neuroinflammation and protein aggregation co-localize across the frontotemporal dementia spectrum (2020) · cites this paper as Mackenzie et al., 2010
The paper’s main text (page unavailable) says most FTLD cases have tau- or TDP-43-containing inclusions and classifies subtypes by characteristic molecular pathology, supporting the aggregation and diagnostic-classification point. It does not identify protein misfolding as a diagnostic criterion, and it cautions that the classification does not presume the proteins have a primary pathogenic role. (LLM, conf=0.84)
Experimental — accuracy not yet measuredOlder studies that reported cases with dementia and no other known pathologic substrate lacked insights into more recently defined pathologies, such as those with aberrant TDP-43, valosin-containing protein, and fused in sarcoma inclusions.
“Older studies that reported cases with dementia and no other known pathologic substrate lacked insights into more recently defined pathologies, such as those with aberrant TDP-43, valosin-containing protein, and fused in sarcoma inclusions (37, 167).”
From Correlation of Alzheimer Disease Neuropathologic Changes With Cognitive Status: A Review of the Literature (2012) · cites this paper as [167]
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- Authors
- Mackenzie, I. R. A., Neumann, M., Bigio, E. H., Cairns, N. J., Alafuzoff, I., Kril, J., Kovacs, G. G., Ghetti, B., Halliday, G., Holm, I. E., Ince, P. G., Kamphorst, W., Revesz, T., Rozemuller, A. J. M., Kumar-Singh, S., Akiyama, H., Baborie, A., Spina, S., Dickson, D. W., Trojanowski, J. Q., & Mann, D. M. A.
- Affiliations
- University of British Columbia (CA)
- University of Zurich (CH)
- Northwestern University (US)
- Washington University in St. Louis (US)
- Uppsala University (SE)
- The University of Sydney (AU)
- Indiana University School of Medicine (US)
- UNSW Sydney (AU)
- Aarhus University (DK)
- University of Sheffield (GB)
- Vrije Universiteit Amsterdam (NL)
- Tokyo Institute of Psychiatry (JP)
- Walton Centre (GB)
- Mayo Clinic (US)
- University of Pennsylvania (US)
- University of Manchester (GB)
- Corresponding author
- Mackenzie, Ian R. A.
- ORCID
- Venue
- Acta Neuropathologica
- Published
- 2009-11-19
- Volume
- 119
- Issue
- 1
- Pages
- 1-4
- ISSN
- 0001-6322 (ISSN-L)
- 1432-0533
- PMC
- PMC2799633
- OpenAlex
- W2067439875
- Cited by
- 1,012
- References
- 15
- Citations by year
- 2026: 36 · 2025: 44 · 2024: 52 · 2023: 41 · 2022: 64 · 2021: 60 · 2020: 62 · 2019: 53 · 2018: 55 · 2017: 51 · 2016: 63 · 2015: 76 · 2014: 56 · 2013: 71 · 2012: 78
- FWCI
- 46.59
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- cc-by-nc
- Locations
- Acta Neuropathologica (publishedVersion, cc-by-nc)
- Europe PMC (PubMed Central) (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Research Explorer (The University of Manchester) (publishedVersion)
- White Rose Research Online (University of Leeds, The University of Sheffield, University of York) (acceptedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (submittedVersion)
- Digital Academic REpository of VU University Amsterdam (Vrije Universiteit Amsterdam) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- IUScholarWorks (Indiana University) (submittedVersion, cc-by-nc)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Publisher
- Zurich Open Repository and Archive (University of Zurich)
- Funders
- Japan Society for the Promotion of Science (21591536)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- frontotemporal lobar degeneration, nomenclature, disease classification, tauopathy, TDP-43, corticobasal degeneration, Pick's disease, progressive supranuclear palsy
- MeSH terms
- Inclusion Bodies, DNA-Binding Proteins, Humans, Intermediate Filaments, Terminology as Topic, tau Proteins, RNA-Binding Protein FUS, Ubiquitinated Proteins, Frontotemporal Lobar Degeneration
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