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Record W4403970975 · doi:10.1101/2024.10.28.24314051

Exome analysis of 22,319 individuals links extremely rare CNVs and 22q11.21 dosage to Alzheimer’s risk

2024· preprint· en· W4403970975 on OpenAlexaff
Olivier Quenez, Catherine Schramm, Kévin Cassinari, Aude Nicolas, J H M Groeneveld, Guillaume Huguet, Benjamin Grenier‐Boley, Marc Hulsman, Jakub Hort, Holger Hummerich, M. Arfan Ikram, M. Kamran Ikram, Martin Ingelsson, Iris E. Jansen, Amit Kawalia, Robert Kraaij, Patrick G. Kehoe, M Lathrop, Morgane Lacour, Afina W. Lemstra, Alberto Lleó, Lauren Luckcuck, Marcel M. A. M. Mannens, Iain Marshall, Carlo Masullo, Simon Mead, Patrizia Mecocci, Alexandre de Mendonça, Alun Meggy, Shima Mehrabian, Merel O. Mol, Kevin Morgan, Alexandre Morin, Benedetta Nacmias, Penny J. Norsworthy, Magda Tsolaki, Sébastien Jacquemont, Jean‐Charles Lambert, Sven J. van der Lee, Camille Charbonnier, Gaël Nicolas

Bibliographic record

VenuemedRxiv · 2024
Typepreprint
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenomics and Rare Diseases
Canadian institutionsUniversité de MontréalMcGill University and Génome Québec Innovation CentreCentre Hospitalier Universitaire Sainte-Justine
FundersNederlandse Organisatie voor Wetenschappelijk OnderzoekZonMw
KeywordsExome sequencingDiseaseExomeCopy-number variationGeneticsMedicineAssociation (psychology)BioinformaticsBiologyComputational biologyPsychologyGeneInternal medicineMutationGenome

Abstract

fetched live from OpenAlex

Abstract Copy number variants (CNVs), defined as deletions or duplications of genomic segments >100 bp, are major contributors to human disease, yet their role in non-monogenic Alzheimer disease (AD) remains poorly characterized. We analyzed rare CNVs from 22,319 exomes, including 4,150 early-onset AD (EOAD), 8,519 late-onset AD and 9,650 unaffected controls. EOAD cases showed increased burdens of rare CNVs, particularly rare deletions within AD-related genes. Loss-of-function analyses implicated deletions in ABCA1 and ABCA7 and identified CTSB as a candidate locus . Exome-wide gene-level dosage analysis highlighted 18 genes across five loci , including chr22q11.21, where deletions were restricted to EOAD and duplications enriched in controls. Replication in 33,992 cases and 362,305 controls confirmed AD-risk reduction in 22q11.21 duplication carriers (mega-analysis dosage OR(SCARF2)=0.34, p=5.52×10 -7 ). SCARF2 overexpression increased amyloid-β uptake in vitro , supporting a functional link. These findings highlight CNVs as contributors to AD risk and identify 22q11.21 dosage as a strong genetic determinant.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.004
Threshold uncertainty score0.015

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.001
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0040.000

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 head0.021
GPT teacher head0.278
Teacher spread0.257 · 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 source (direct Gemma or distilled Codex), 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

Citations0
Published2024
Admission routes1
Has abstractyes

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