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Record W7162120152 · doi:10.82308/22698

Toward a molecular description of proteus syndrome

2004· dissertation· en· W7162120152 on OpenAlexaboutno aff
Isabelle Thiffault

Bibliographic record

Venuenot available
Typedissertation
Languageen
FieldMedicine
TopicVascular Malformations and Hemangiomas
Canadian institutionsnot available
Fundersnot available
KeywordsProteus syndromeSomatic cellGermline mutationProteusMutationMicrosatelliteDNA

Abstract

fetched live from OpenAlex

Proteus Syndrome is a rare overgrowth syndrome in which tumors are a prominent feature. Proteus syndrome comprises an association of asymmetrical gigantism, verrucous epidermal naevi, vascular malformations, hamartomas and hyperostosis. There is no known molecular basis for this overgrowth syndrome but several reports have suggested abnormalities of chromosome 1 could play a role and the abnormal regulation of growth factors could also be important. We obtained paired and unpaired DNA samples from seven cases of Proteus syndrome from Montreal and Greenwood Genetics Center, South Carolina. In all analyses, we compared simultaneously DNA from affected and unaffected areas from these children. Direct sequencing was used to look at somatic mutation or other alterations in growth, apoptosis or tumor suppressor genes, such as PTEN, GPC3 and CDKN1C. A genome-wide, 10cM 388 marker microsatellite screen were performed to uncover putative somatic genomic microdeletions or other rearrangements by comparing the allelotype of the affected and unaffected tissues from Proteus syndrome patients.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.001
Threshold uncertainty score0.005

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.000
Science and technology studies0.0000.001
Scholarly communication0.0010.001
Open science0.0010.000
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0010.001

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 head0.022
GPT teacher head0.263
Teacher spread0.242 · 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 source (direct Gemma or distilled Codex), 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
Published2004
Admission routes1
Has abstractyes

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