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Record W4324117092 · doi:10.1016/j.gimo.2023.100360

P332: A homozygous POU3F2 polyproline tract expansion is associated with severe psychomotor delay, developmental and epileptic encephalopathy, and ichthyosis

2023· article· en· W4324117092 on OpenAlexaff
Camille Tremblay‐Laganière, Sophie Ehresmann, Alina Levtova, Julie Gauthier, He Fu, Robert J. McEvilly, Michael G. Rosenfeld, Elisabeth Simard‐Tremblay, Isabelle DeBie, Jean‐François Soucy, Jacques L. Michaud, Emmanuelle Lemyre, Philippe M. Campeau

Bibliographic record

VenueGenetics in Medicine Open · 2023
Typearticle
Languageen
FieldMedicine
TopicDiet and metabolism studies
Canadian institutionsUniversité de MontréalMcGill University Health CentreMcGill UniversityCentre Hospitalier de l’Université de MontréalCentre Hospitalier Universitaire Sainte-Justine
Fundersnot available
KeywordsPsychomotor learningMedicineIchthyosisEpilepsyEncephalopathyPsychomotor retardationMicrocephalyPediatricsDermatologyInternal medicinePsychiatryPathology

Abstract

fetched live from OpenAlex

POU3F2 is an essential transcription factor involved in neurogenesis, cortical neural migration, and hypothalamic neurons’ late differentiation. POU3F2 function has been well studied in animal models in which complete knockout of POU3F2 causes abnormal neurogenesis and early mortality. Specific loss of highly conserved homopolymeric amino acid repeats in POU3F2 in mice was linked to behavioral anomalies, cognitive impairment and reduced hippocampal neurogenesis. Still, studying the importance of POU3F2 in human brain development is limited by the fact that there are only a few reports of POU3F2-related disease (dominant loss of function) in humans.

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)0.000
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 score0.168
Threshold uncertainty score0.875

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.040
GPT teacher head0.318
Teacher spread0.278 · 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

Citations0
Published2023
Admission routes1
Has abstractyes

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