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Record W4392616567 · doi:10.1016/j.gimo.2024.101557

P652: High-yield diagnostic testing of FGF14 repeat expansion in cerebellar ataxia across the North American population

2024· article· en· W4392616567 on OpenAlexaff
M. Bruce Forrest, Megan Delfeld, Alexander Gary, April Franco, Sanjukta Tawde, Malia Triebold, Widad Abou-Chaar, Vikram G. Shakkottai, Sharan R. Srinivasan, Khalaf Bushara, Bernard Brais, David Pellerin, Christopher M. Gómez, Soma Das

Bibliographic record

VenueGenetics in Medicine Open · 2024
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsMontreal Neurological Institute and Hospital
Fundersnot available
KeywordsAtaxiaCerebellar ataxiaPopulationYield (engineering)MedicinePsychologyNeurosciencePhysics

Abstract

fetched live from OpenAlex

Heterozygous GAA repeat expansions in intron 1 of the FGF14 gene have been recently described in an autosomal dominant form of adult-onset cerebellar ataxia (SCA27B). This expansion has been shown to occur in a high proportion of individuals particularly with late-onset cerebellar ataxia and has been seen in several different subpopulations. While normal individuals have a polymorphic number of repeats in this region (<250 repeats), repeat sizes greater than 300 are significantly associated with disease, and repeat sizes between 250 and 300 are likely pathogenic with reduced penetrance.

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)0.008
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMetaresearch
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.080
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.008
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.002
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0010.001
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 head0.071
GPT teacher head0.352
Teacher spread0.282 · 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.

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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