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Record W2939216510 · doi:10.4155/bio-2018-0306

Distribution of Heparan Sulfate and Dermatan Sulfate in Mucopolysaccharidosis Type Ii Mouse Tissues Pre- and Post-Enzyme-Replacement Therapy Determined By Uplc–Ms/Ms

2019· article· en· W2939216510 on OpenAlex

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.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueBioanalysis · 2019
Typearticle
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsUniversité de Sherbrooke
FundersÁltalános Orvostudományi Kar, Pécsi Tudományegyetem
KeywordsMucopolysaccharidosis type IIDermatan sulfateEnzyme replacement therapyHeparan sulfateHunter syndromeChemistryEnzymeMucopolysaccharidosisGlycosaminoglycanChromatographyEnzyme assayBiochemistryMolecular biologyInternal medicineMedicineBiologyDisease

Abstract

fetched live from OpenAlex

Aim: Mucopolysaccharidosis type II (MPS II) is a lysosomal storage disorder caused by a deficiency of the iduronate-2-sulfatase enzyme leading to the accumulation of heparan sulfate (HS) and dermatan sulfate (DS) in organs and biological fluids. enzyme-replacement therapy is available for affected patients. Results/methodology: A 6-min UPLC–MS/MS method was developed/validated for HS and DS quantification in mouse tissues and biological fluids with high accuracy and precision. In MPS II mice, HS was more abundant than DS. 8-week enzyme-replacement therapy significantly reduced HS and DS levels in all matrices, except the brain. These reduced levels were maintained over a 16-week extended treatment period. Conclusion: The devised method is sensitive, robust and useful for the evaluation of biomarker distribution in MPS II mice.

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 categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.107
Threshold uncertainty score0.770

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
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 head0.012
GPT teacher head0.285
Teacher spread0.273 · 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