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Record W4285394094 · doi:10.1212/nxg.0000000000000678

Questioning the Association of the <i>STMN2</i> Dinucleotide Repeat With Amyotrophic Lateral Sclerosis

2022· article· en· W4285394094 on OpenAlex
Jay P. Ross, Fulya Akçimen, Calwing Liao, Dan Spiegelman, Ben Weisburd, Nicolas Dupré, Patrick A. Dion, Guy A. Rouleau, Sali M.K. Farhan

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueNeurology Genetics · 2022
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsUniversité du Québec à MontréalMontreal Neurological Institute and Hospital
Fundersnot available
KeywordsAmyotrophic lateral sclerosisGeneticsCohortBiologyGeneGenetic associationGenotypeSingle-nucleotide polymorphismBioinformaticsMedicineInternal medicineDisease

Abstract

fetched live from OpenAlex

<h3>Objectives</h3> Recently, the number of dinucleotide CA repeats in an intron of the <i>STMN2</i> gene was reported to be associated with an increased risk for amyotrophic lateral sclerosis (ALS). Therefore, we sought to replicate this observation in an independent group of ALS patients and a much larger control group. <h3>Methods</h3> Here, we used whole-genome sequencing and tested the <i>STMN2</i> CA repeat in a case-control cohort of the European genetic background and in genomes from various populations in the gnomAD cohort to attempt to replicate this proposed association. <h3>Results</h3> We find that repeats well above the previously reported pathogenic threshold of 19 are commonly observed in unaffected individuals across different populations. Furthermore, we did not observe an association between longer <i>STMN2</i> CA repeats and ALS phenotype. <h3>Discussion</h3> In summary, our results do not support a role of <i>STMN2</i> CA repeats toward ALS risk. As TDP-43 aggregation is central to ALS pathogenesis, lowered expression of <i>STMN2</i> could be used as a biomarker for ALS. Therefore, a variant associated both with the risk for ALS and the level of <i>STMN2</i> expression would be clinically useful. However, for a variant to be actionable, it must be strongly replicated in independent cohorts and exceed the rigorous statistical thresholds applied.

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.

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 score0.058
Threshold uncertainty score0.371

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 integrity0.0000.001
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 head0.016
GPT teacher head0.229
Teacher spread0.213 · 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