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Record W4392588909 · doi:10.1016/j.gimo.2024.101092

P195: Investigating the contribution of an intronic expansion repeat in FGF14 as a genetic cause of late-onset ataxia

2024· article· en· W4392588909 on OpenAlexaff
Alexanne Cuillerier, Layla Mackay, Erika Wall, Giulia Gobbo, Luke Seldenthuis, Wendy Mears, Alexandre White‐Brown, Laura Larrigan, David A. Dyment, Kym M. Boycott

Bibliographic record

VenueGenetics in Medicine Open · 2024
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsChildren's Hospital of Eastern Ontario
Fundersnot available
KeywordsAtaxiaTrinucleotide repeat expansionGeneticsBiologyNeuroscienceGeneAllele

Abstract

fetched live from OpenAlex

Ataxias are a clinically and genetically heterogeneous group of progressive neurodegenerative diseases. Patients present anywhere from childhood to adulthood with prominent cerebellum atrophy primarily leading to unsteady gait, clumsiness, eye movement anomalies, and dysarthria. Ataxias can be sporadic or transmitted in a recessive or dominant fashion, making molecular diagnosis extremely challenging; only 25% of patients have causative variants identified. Many inherited forms of ataxias are associated with expansion repeats of tri-nucleotides in both exonic and intronic regions of genes, yielding unstable expanded alleles.

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)0.001
metaresearch head score (Gemma)0.002
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Bench or experimental · Consensus signal: Bench or experimental
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.103
Threshold uncertainty score0.609

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.001
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0010.000
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 head0.057
GPT teacher head0.361
Teacher spread0.304 · 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 designBench or experimental
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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