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Record W2152426559

Tuberous Sclerosis Complex: mutations, functions and phenotypes

2005· article· en· W2152426559 on OpenAlex
Őzgür Sancak

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.

fundA Canadian funder is recorded on the work.
no affNo Canadian affiliation: this work is invisible to an affiliation-only frame.
No Canadian affiliation. An affiliation-only frame, the usual design, would never have seen this work. It is one of the works that make the case for inverting the frame.

Bibliographic record

VenueRePub (Erasmus University Rotterdam) · 2005
Typearticle
Languageen
FieldMedicine
TopicTuberous Sclerosis Complex Research
Canadian institutionsnot available
FundersCanadian Institutes of Health ResearchNational Institutes of HealthNederlandse Organisatie voor Wetenschappelijk OnderzoekBelgian Federal Science Policy OfficeSavoy Foundation
KeywordsTSC1TSC2Tuberous sclerosisMutationPhenotypeBiologyGeneticsGeneMutation testingMolecular biologyMedicinePathologyPI3K/AKT/mTOR pathway
DOInot available

Abstract

fetched live from OpenAlex

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterised by the \ndevelopment of hamartomas in multiple organs and tissues. TSC is caused by mutations in \neither the TSC1 or TSC2 gene. We searched for mutations in both genes in a cohort of 490 \npatients diagnosed with or suspected of having TSC using a combination of denaturing gradient \ngel electrophoresis, single-strand conformational polymorphism, direct sequencing, fluorescent \nin situ hybridisation and Southern blotting. We identified pathogenic mutations in 362 patients, \na mutation detection rate of 74%. Of these 362 patients, 276 had a definite clinical diagnosis of \nTSC and in these patients 235 mutations were identified, a mutation detection rate of 85%. The \nratio of TSC2:TSC1 mutations was 3.4:1. In our cohort, both TSC1 mutations and mutations in \nfamilial TSC2 cases were associated with phenotypes less severe than de novo TSC2 mutations. \nInterestingly, consistent with other studies, the phenotypes of the patients in which no mutation \nwas identified were, overall, less severe than those of patients with either a known TSC1 or TSC2 \nmutation.

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 categoriesInsufficient payload (model declined to judge)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.558
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

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

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 head0.050
GPT teacher head0.255
Teacher spread0.205 · 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