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Record W2107614818 · doi:10.1002/cbic.200890061

Cover Picture: Identification of Pharmacological Chaperones for Gaucher Disease and Characterization of Their Effects on β‐Glucocerebrosidase by Hydrogen/Deuterium Exchange Mass Spectrometry (ChemBioChem 16/2008)

2008· paratext· en· W2107614818 on OpenAlexaff
Michael B. Tropak, G. Kornhaber, Brigitte Rigat, Gustavo Maegawa, Justin Buttner, Jan Blanchard, Cecilia Murphy, Steven Tuske, Stephen J. Coales, Yoshitomo Hamuro, Eric D. Brown, Don J. Mahuran

Bibliographic record

VenueChemBioChem · 2008
Typeparatext
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsUniversity of TorontoMcMaster UniversityHospital for Sick Children
Fundersnot available
KeywordsGlucocerebrosidaseHydrogen–deuterium exchangeChemistryEnzymeSubstrate (aquarium)StereochemistryBiochemistryMass spectrometryBiologyChromatography

Abstract

fetched live from OpenAlex

The cover picture shows the regions (shades of blue) on human lysosomal glucocerebrosidase (green) that undergo a significant reduction in hydrogen–deuterium exchange (denoted by loss of blurriness) upon binding a 2,4-diamino-5-substituted quinazoline (MWP; red). MWP was identified in a high-throughput screen of the Maybridge library of 50 000 small molecule drug-like compounds (grey structures) for inhibitors. The confocal images immediately below the 3D structures of the enzyme are patient fibrobroblasts homozygous for the common N370S Gaucher disease type I allele, stained with an antibody against glucocerebrosidase (green); these show increased levels of the enzyme in MWP treated (+MWP) cells in comparison with untreated control (−MWP) cells. This study has identified pharmacological chaperones (PC) that are not based on the structure of the glycolipid substrate of the enzyme. These new compounds increase the repertoire of chemical frameworks available for the construction of efficient PCs for the treatment of Gaucher disease. For more details, see the article by M. B. Tropak, D. J. Mahuran et al. on p. 2650 ff.

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)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
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 score0.043
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0010.000
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 head0.017
GPT teacher head0.282
Teacher spread0.265 · 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 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

Citations1
Published2008
Admission routes1
Has abstractyes

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