Literature DB >> 16705058

Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats.

Renée P Kinman1, Takhar Kasumov, Kathryn A Jobbins, Katherine R Thomas, Jillian E Adams, Lisa N Brunengraber, Gerd Kutz, Wolf-Ulrich Brewer, Charles R Roe, Henri Brunengraber.   

Abstract

A new chronic treatment for inherited disorders of long-chain fatty acid oxidation involves administering up to one-third of dietary calories as triheptanoin, a medium-odd-chain triglyceride (Roe CR, Sweetman L, Roe DS, David F, and Brunengraber H. J Clin Invest 110: 259-269, 2002). Heptanoate and C(5)-ketone bodies derived from its partial oxidation in liver are precursors of anaplerotic propionyl-CoA in peripheral tissues. It was hypothesized that increasing anaplerosis in peripheral tissues would boost energy production. In the present study, we tested the potential of a triheptanoin emulsion as an intravenous nutrient. Normal rats were infused with triheptanoin intravenously or intraduodenally at up to 40% of caloric requirement. The blood concentration ratio (heptanoate/C(5)-ketone bodies) was high with intravenous and low with intraduodenal triheptanoin infusion. During intravenous infusion of triheptanoin, lipolysis was stimulated but appeared compensated by fatty acid reesterification. During intraduodenal infusion of triheptanoin, lipolysis was not stimulated. Our data support the hypothesis that intravenous triheptanoin could be used to treat decompensated patients with long-chain fatty acid oxidation disorders.

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Year:  2006        PMID: 16705058     DOI: 10.1152/ajpendo.00366.2005

Source DB:  PubMed          Journal:  Am J Physiol Endocrinol Metab        ISSN: 0193-1849            Impact factor:   4.310


  25 in total

1.  Dietary Treatments and New Therapeutic Perspective in GLUT1 Deficiency Syndrome.

Authors:  Pierangelo Veggiotti; Valentina De Giorgis
Journal:  Curr Treat Options Neurol       Date:  2014-05       Impact factor: 3.598

2.  Comparative study of serine-plasmalogens in human retina and optic nerve: identification of atypical species with odd carbon chains.

Authors:  Kornél Nagy; Viral Vishnuprasad Brahmbhatt; Olivier Berdeaux; Lionel Bretillon; Frédéric Destaillats; Niyazi Acar
Journal:  J Lipid Res       Date:  2012-01-19       Impact factor: 5.922

3.  Triheptanoin alters [U-13C6]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.

Authors:  Tanya McDonald; Mark P Hodson; Ilya Bederman; Michelle Puchowicz; Karin Borges
Journal:  J Cereb Blood Flow Metab       Date:  2019-03-19       Impact factor: 6.200

Review 4.  Modification of Astrocyte Metabolism as an Approach to the Treatment of Epilepsy: Triheptanoin and Acetyl-L-Carnitine.

Authors:  Mussie Ghezu Hadera; Tanya McDonald; Olav B Smeland; Tore W Meisingset; Haytham Eloqayli; Saied Jaradat; Karin Borges; Ursula Sonnewald
Journal:  Neurochem Res       Date:  2015-10-03       Impact factor: 3.996

5.  Role of mitochondrial acyl-CoA dehydrogenases in the metabolism of dicarboxylic fatty acids.

Authors:  Sivakama S Bharathi; Yuxun Zhang; Zhenwei Gong; Radhika Muzumdar; Eric S Goetzman
Journal:  Biochem Biophys Res Commun       Date:  2020-04-29       Impact factor: 3.575

6.  Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplement.

Authors:  Juan M Pascual; Peiying Liu; Deng Mao; Dorothy I Kelly; Ana Hernandez; Min Sheng; Levi B Good; Qian Ma; Isaac Marin-Valencia; Xuchen Zhang; Jason Y Park; Linda S Hynan; Peter Stavinoha; Charles R Roe; Hanzhang Lu
Journal:  JAMA Neurol       Date:  2014-10       Impact factor: 18.302

Review 7.  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

8.  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

9.  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

10.  Sports in LCHAD Deficiency: Maximal Incremental and Endurance Exercise Tests in a 13-Year-Old Patient with Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency (LCHADD) and Heptanoate Treatment.

Authors:  D Karall; G Mair; U Albrecht; K Niedermayr; T Karall; W Schobersberger; S Scholl-Bürgi
Journal:  JIMD Rep       Date:  2014-07-06
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