Literature DB >> 7900792

Metabolism of R-beta-hydroxypentanoate and of beta-ketopentanoate in conscious dogs.

J Leclerc1, C Des Rosiers, J A Montgomery, J Brunet, L Ste-Marie, M W Reider, C A Fernandez, L Powers, F David, H Brunengraber.   

Abstract

R-beta-Hydroxypentanoate and beta-ketopentanoate are homologues of physiological ketone bodies R-beta-hydroxybutyrate and acetoacetate. They derive from the oxidation in liver of the R-moiety of R,S-1,3-pentanediol, a potential nutrient. This report documents the metabolism of R-beta-hydroxypentanoate and beta-ketopentanoate in conscious dogs. Whether administered by bolus or constant infusion, the two substrates are interconverted and rapidly metabolized. When beta-ketopentanoate was infused at a rate corresponding to 75% of the dog's caloric requirement, the steady-state total plasma concentration of the two substrates was only 1.3 mM. Because the substrates are precursors of propionyl-CoA, we assayed the urinary concentrations of markers of propionic acidemia. Their accumulation was minor compared with what is observed in patients suffering from propionic acidemia. We conclude that, at least during short-term experiments, R-beta-hydroxypentanoate and beta-ketopentanoate are well metabolized in the dog without apparent intolerance to a large supply of propionyl-CoA.

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Year:  1995        PMID: 7900792     DOI: 10.1152/ajpendo.1995.268.3.E446

Source DB:  PubMed          Journal:  Am J Physiol        ISSN: 0002-9513


  10 in total

Review 1.  Anaplerotic molecules: current and future.

Authors:  Henri Brunengraber; Charles R Roe
Journal:  J Inherit Metab Dis       Date:  2006 Apr-Jun       Impact factor: 4.982

2.  Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver.

Authors:  Shuang Deng; Guo-Fang Zhang; Takhar Kasumov; Charles R Roe; Henri Brunengraber
Journal:  J Biol Chem       Date:  2009-08-08       Impact factor: 5.157

Review 3.  Triheptanoin--a medium chain triglyceride with odd chain fatty acids: a new anaplerotic anticonvulsant treatment?

Authors:  Karin Borges; Ursula Sonnewald
Journal:  Epilepsy Res       Date:  2011-08-19       Impact factor: 3.045

4.  Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models.

Authors:  Sarah Willis; James Stoll; Lawrence Sweetman; Karin Borges
Journal:  Neurobiol Dis       Date:  2010-08-04       Impact factor: 5.996

5.  Development and validation of a LC-MS/MS method for quantitation of 3-hydroxypentanoic acid and 3-oxopentanoic acid in human plasma and its application to a clinical study of glucose transporter type I deficiency (G1D) syndrome.

Authors:  Raja Reddy Kallem; Sharon Primeaux; Adrian Avila; Juan M Pascual; William C Putnam
Journal:  J Pharm Biomed Anal       Date:  2021-08-27       Impact factor: 3.935

6.  Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.

Authors:  Charles R Roe; Lawrence Sweetman; Diane S Roe; France David; Henri Brunengraber
Journal:  J Clin Invest       Date:  2002-07       Impact factor: 14.808

7.  Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile.

Authors:  Lei Gu; Guo-Fang Zhang; Rajan S Kombu; Frederick Allen; Gerd Kutz; Wolf-Ulrich Brewer; Charles R Roe; Henri Brunengraber
Journal:  Am J Physiol Endocrinol Metab       Date:  2009-11-10       Impact factor: 4.310

8.  Heptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brain.

Authors:  Isaac Marin-Valencia; Levi B Good; Qian Ma; Craig R Malloy; Juan M Pascual
Journal:  J Cereb Blood Flow Metab       Date:  2012-10-17       Impact factor: 6.200

9.  Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy.

Authors:  C R Roe; B-Z Yang; H Brunengraber; D S Roe; M Wallace; B K Garritson
Journal:  Neurology       Date:  2008-07-22       Impact factor: 9.910

10.  Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.

Authors:  Charles R Roe; Henri Brunengraber
Journal:  Mol Genet Metab       Date:  2015-10-24       Impact factor: 4.797

  10 in total

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