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Record W2097717784 · doi:10.1093/hmg/dds161

Evidence for a role of the rare p.A152T variant in MAPT in increasing the risk for FTD-spectrum and Alzheimer's diseases

2012· article· en· W2097717784 on OpenAlexaff
Giovanni Coppola, Subashchandrabose Chinnathambi, Jason JiYong Lee, Beth A. Dombroski, Matt Baker, Alexandra I. Soto‐Ortolaza, Suzee E. Lee, Eric Klein, Alden Huang, Renee Sears, Jessica Lane, Anna M. Karydas, Robert O. Kenet, Jacek Biernat, Li San Wang, Carl W. Cotman, Charles DeCarli, Allan I. Levey, John M. Ringman, Mario F. Mendez, Helena C. Chui, Isabelle Le Ber, Alexis Brice, Michelle K. Lupton, Elisavet Preza, Simon Lovestone, John Powell, Neill R. Graff‐Radford, Ronald Petersen, Bradley F. Boeve, Carol F. Lippa, Eileen H. Bigio, Ian R. Mackenzie, Elizabeth Finger, Andrew Kertesz, Richard J. Caselli, Marla Gearing, Jorge L. Juncos, Bernardino Ghetti, Salvatore Spina, Yvette Bordelon, Wallace W. Tourtellotte, Matthew P. Frosch, Jean Paul Vonsattel, Chris Zarow, Thomas G. Beach, Roger L. Albin, Andrew P. Lieberman, Virginia M. Lee, John Q. Trojanowski, Vivianna M. Van Deerlin, Thomas D. Bird, Douglas Galasko, Eliezer Masliah, Charles L. White, Juan C. Troncoso, Didier Hannequin, Adam L. Boxer, Michael D. Geschwind, Satish Kumar, Eva‐Maria Mandelkow, Zbigniew K. Wszołek, Ryan J. Uitti, Dennis W. Dickson, Jonathan L. Haines, Richard Mayeux, Margaret A. Pericak‐Vance, Lindsay A. Farrer, Owen A. Ross, Rosa Rademakers, Bruce L. Miller, Eckhard Mandelkow, Daniel H. Geschwind

Bibliographic record

VenueHuman Molecular Genetics · 2012
Typearticle
Languageen
FieldMedicine
TopicAlzheimer's disease research and treatments
Canadian institutionsWestern UniversityUniversity of British Columbia
FundersNational Institute on AgingMedical Research CouncilSchool of Medicine, Emory UniversityNational Institutes of HealthNational Institute of Mental HealthNorthwestern UniversityArizona Biomedical Research CommissionNational Center for Advancing Translational SciencesNational Human Genome Research InstituteWellcome TrustAgence Nationale de la RechercheNational Institute of Neurological Disorders and StrokeNational Institute for Health and Care ResearchNational Center for Research ResourcesUniversity of PennsylvaniaCurePSPEuropean CommissionLouisiana State UniversityArizona Department of Health ServicesEmory University
KeywordsProgressive supranuclear palsyCorticobasal degenerationFrontotemporal dementiaTau proteinBiologyAlzheimer's diseaseHaplotypeDementiaDiseaseNeuroscienceGeneticsInternal medicineGeneMedicineAllele

Abstract

fetched live from OpenAlex

Rare mutations in the gene encoding for tau (MAPT, microtubule-associated protein tau) cause frontotemporal dementia-spectrum (FTD-s) disorders, including FTD, progressive supranuclear palsy (PSP) and corticobasal syndrome, and a common extended haplotype spanning across the MAPT locus is associated with increased risk of PSP and Parkinson's disease. We identified a rare tau variant (p.A152T) in a patient with a clinical diagnosis of PSP and assessed its frequency in multiple independent series of patients with neurodegenerative conditions and controls, in a total of 15 369 subjects. Tau p.A152T significantly increases the risk for both FTD-s (n = 2139, OR = 3.0, CI: 1.6-5.6, P = 0.0005) and Alzheimer's disease (AD) (n = 3345, OR = 2.3, CI: 1.3-4.2, P = 0.004) compared with 9047 controls. Functionally, p.A152T (i) decreases the binding of tau to microtubules and therefore promotes microtubule assembly less efficiently; and (ii) reduces the tendency to form abnormal fibers. However, there is a pronounced increase in the formation of tau oligomers. Importantly, these findings suggest that other regions of the tau protein may be crucial in regulating normal function, as the p.A152 residue is distal to the domains considered responsible for microtubule interactions or aggregation. These data provide both the first genetic evidence and functional studies supporting the role of MAPT p.A152T as a rare risk factor for both FTD-s and AD and the concept that rare variants can increase the risk for relatively common, complex neurodegenerative diseases, but since no clear significance threshold for rare genetic variation has been established, some caution is warranted until the findings are further replicated.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.166
Threshold uncertainty score0.365

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.052
GPT teacher head0.337
Teacher spread0.286 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations236
Published2012
Admission routes1
Has abstractyes

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