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Record W3126472171 · doi:10.1002/jimd.12365

Enantiomer‐specific pharmacokinetics of D,L‐3‐hydroxybutyrate: Implications for the treatment of multiple <scp>acyl‐CoA</scp> dehydrogenase deficiency

2021· article· en· W3126472171 on OpenAlexaff
Willemijn J. van Rijt, Johan L.K. Van Hove, Frédéric M. Vaz, Rick Havinga, Derk P. Allersma, Tanja R. Zijp, Jirair K. Bedoyan, M. Rebecca Heiner‐Fokkema, Dirk‐Jan Reijngoud, Michael T. Geraghty, Ronald J. A. Wanders, Maaike H. Oosterveer, Terry G. J. Derks

Bibliographic record

VenueJournal of Inherited Metabolic Disease · 2021
Typearticle
Languageen
FieldMedicine
TopicDiet and metabolism studies
Canadian institutionsChildren's Hospital of Eastern Ontario
FundersUniversitair Medisch Centrum GroningenRijksuniversiteit Groningen
KeywordsCmaxPharmacokineticsInternal medicineEndocrinologyMetabolismKetone bodiesMedicineEnantiomerChemistryStereochemistry

Abstract

fetched live from OpenAlex

Abstract D,L‐3‐hydroxybutyrate (D,L‐3‐HB, a ketone body) treatment has been described in several inborn errors of metabolism, including multiple acyl‐CoA dehydrogenase deficiency (MADD; glutaric aciduria type II). We aimed to improve the understanding of enantiomer‐specific pharmacokinetics of D,L‐3‐HB. Using UPLC‐MS/MS, we analyzed D‐3‐HB and L‐3‐HB concentrations in blood samples from three MADD patients, and blood and tissue samples from healthy rats, upon D,L‐3‐HB salt administration (patients: 736‐1123 mg/kg/day; rats: 1579‐6317 mg/kg/day of salt‐free D,L‐3‐HB). D,L‐3‐HB administration caused substantially higher L‐3‐HB concentrations than D‐3‐HB. In MADD patients, both enantiomers peaked at 30 to 60 minutes, and approached baseline after 3 hours. In rats, D,L‐3‐HB administration significantly increased C max and AUC of D‐3‐HB in a dose‐dependent manner (controls vs ascending dose groups for C max : 0.10 vs 0.30‐0.35‐0.50 mmol/L, and AUC: 14 vs 58‐71‐106 minutes*mmol/L), whereas for L‐3‐HB the increases were significant compared to controls, but not dose proportional ( C max : 0.01 vs 1.88‐1.92‐1.98 mmol/L, and AUC: 1 vs 380‐454‐479 minutes*mmol/L). L‐3‐HB concentrations increased extensively in brain, heart, liver, and muscle, whereas the most profound rise in D‐3‐HB was observed in heart and liver. Our study provides important knowledge on the absorption and distribution upon oral D,L‐3‐HB. The enantiomer‐specific pharmacokinetics implies differential metabolic fates of D‐3‐HB and L‐3‐HB.

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)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Bench or experimental · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.768
Threshold uncertainty score0.697

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.001
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 head0.043
GPT teacher head0.317
Teacher spread0.275 · 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 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

Citations22
Published2021
Admission routes1
Has abstractyes

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