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Maximum dose, safety, tolerability and ketonemia after triheptanoin in glucose transporter type 1 deficiency (G1D).
Málaga I, Avila A, Primeaux S, Kallem RR, Roe CR, Putnam WC, Park JY, Shinnar S, Ahn C, Pascual JM
Sci Rep, 13(1):3465, 01 Mar 2023
Cited by: 1 article | PMID: 36859467 | PMCID: PMC9977760
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Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.
Roe CR, Brunengraber H
Mol Genet Metab, 116(4):260-268, 24 Oct 2015
Cited by: 40 articles | PMID: 26547562 | PMCID: PMC4712637
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Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice.
Park MJ, Aja S, Li Q, Degano AL, Penati J, Zhuo J, Roe CR, Ronnett GV
PLoS One, 9(10):e109527, 09 Oct 2014
Cited by: 41 articles | PMID: 25299635 | PMCID: PMC4192301
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Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplement.
Pascual JM, Liu P, Mao D, Kelly DI, Hernandez A, Sheng M, Good LB, Ma Q, Marin-Valencia I, Zhang X, Park JY, Hynan LS, Stavinoha P, Roe CR, Lu H
JAMA Neurol, 71(10):1255-1265, 01 Oct 2014
Cited by: 58 articles | PMID: 25110966 | PMCID: PMC4376124
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Systemic metabolic abnormalities in adult-onset acid maltase deficiency: beyond muscle glycogen accumulation.
Pascual JM, Roe CR
JAMA Neurol, 70(6):756-763, 01 Jun 2013
Cited by: 11 articles | PMID: 23609947
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Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways.
Roe CR, Bottiglieri T, Wallace M, Arning E, Martin A
Mol Genet Metab, 101(2-3):246-252, 06 Jul 2010
Cited by: 25 articles | PMID: 20655781
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Pyruvate carboxylase deficiency: mechanisms, mimics and anaplerosis.
Marin-Valencia I, Roe CR, Pascual JM
Mol Genet Metab, 101(1):9-17, 09 Jun 2010
Cited by: 64 articles | PMID: 20598931
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Effect of dietary fats with odd or even numbers of carbon atoms on metabolic response and muscle damage with exercise in Quarter Horse-type horses with type 1 polysaccharide storage myopathy.
Borgia LA, Valberg SJ, McCue ME, Pagan JD, Roe CR
Am J Vet Res, 71(3):326-336, 01 Mar 2010
Cited by: 3 articles | PMID: 20187835
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Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver.
Deng S, Zhang GF, Kasumov T, Roe CR, Brunengraber H
J Biol Chem, 284(41):27799-27807, 08 Aug 2009
Cited by: 23 articles | PMID: 19666922 | PMCID: PMC2788830
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Effect of triheptanoin on muscle metabolism during submaximal exercise in horses.
McCue ME, Valberg SJ, Pagan JD, Essén-Gustavsson B, Roe CR
Am J Vet Res, 70(8):1043-1052, 01 Aug 2009
Cited by: 0 articles | PMID: 19645587
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Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile.
Gu L, Zhang GF, Kombu RS, Allen F, Kutz G, Brewer WU, Roe CR, Brunengraber H
Am J Physiol Endocrinol Metab, 298(2):E362-71, 10 Nov 2009
Cited by: 33 articles | PMID: 19903863 | PMCID: PMC2822475
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Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy.
Roe CR, Yang BZ, Brunengraber H, Roe DS, Wallace M, Garritson BK
Neurology, 71(4):260-264, 01 Jul 2008
Cited by: 54 articles | PMID: 18645163 | PMCID: PMC2676979
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Short-chain acyl-CoA dehydrogenase gene mutation (c.319C>T) presents with clinical heterogeneity and is candidate founder mutation in individuals of Ashkenazi Jewish origin.
Tein I, Elpeleg O, Ben-Zeev B, Korman SH, Lossos A, Lev D, Lerman-Sagie T, Leshinsky-Silver E, Vockley J, Berry GT, Lamhonwah AM, Matern D, Roe CR, Gregersen N
Mol Genet Metab, 93(2):179-189, 03 Dec 2007
Cited by: 34 articles | PMID: 18054510
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Choice of oils for essential fat supplements can enhance production of abnormal metabolites in fat oxidation disorders.
Roe CR, Roe DS, Wallace M, Garritson B
Mol Genet Metab, 92(4):346-350, 06 Sep 2007
Cited by: 9 articles | PMID: 17825594
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REMOVED: Short-chain acyl-CoA dehydrogenase gene mutation (319 C>T) presents with clinical heterogeneity and is candidate founder mutation in Ashkenazi Jewish population.
Tein I, Elpeleg O, Ben-Zeev B, Korman SH, Lossos A, Lev D, Lerman-Sagie T, Vockley G, Berry GT, Lamhownah AM, Matern D, Roe CR, Gregersen N
Mol Genet Metab, 17 Jan 2007
Cited by: 1 article | PMID: 17234443
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Anaplerotic molecules: current and future.
Brunengraber H, Roe CR
J Inherit Metab Dis, 29(2-3):327-331, 01 Apr 2006
Cited by: 120 articles | PMID: 16763895
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Variations in IBD (ACAD8) in children with elevated C4-carnitine detected by tandem mass spectrometry newborn screening.
Pedersen CB, Bischoff C, Christensen E, Simonsen H, Lund AM, Young SP, Koeberl DD, Millington DS, Roe CR, Roe DS, Wanders RJ, Ruiter JP, Keppen LD, Stein Q, Knudsen I, Gregersen N, Andresen BS
Pediatr Res, 60(3):315-320, 20 Jul 2006
Cited by: 19 articles | PMID: 16857760
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Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats.
Kinman RP, Kasumov T, Jobbins KA, Thomas KR, Adams JE, Brunengraber LN, Kutz G, Brewer WU, Roe CR, Brunengraber H
Am J Physiol Endocrinol Metab, 291(4):E860-6, 16 May 2006
Cited by: 37 articles | PMID: 16705058
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Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential.
Roe CR, Mochel F
J Inherit Metab Dis, 29(2-3):332-340, 01 Apr 2006
Cited by: 60 articles | PMID: 16763896
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Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts.
Roe DS, Yang BZ, Vianey-Saban C, Struys E, Sweetman L, Roe CR
Mol Genet Metab, 87(1):40-47, 16 Nov 2005
Cited by: 14 articles | PMID: 16297647
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Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy.
Mochel F, DeLonlay P, Touati G, Brunengraber H, Kinman RP, Rabier D, Roe CR, Saudubray JM
Mol Genet Metab, 84(4):305-312, 01 Apr 2005
Cited by: 72 articles | PMID: 15781190
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Respiratory complex II defect in siblings associated with a symptomatic secondary block in fatty acid oxidation.
Gargus JJ, Boyle K, Bocian M, Roe DS, Vianey-Saban C, Roe CR
J Inherit Metab Dis, 26(7):659-670, 01 Jan 2003
Cited by: 7 articles | PMID: 14707514
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Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans.
Nguyen TV, Andresen BS, Corydon TJ, Ghisla S, Abd-El Razik N, Mohsen AW, Cederbaum SD, Roe DS, Roe CR, Lench NJ, Vockley J
Mol Genet Metab, 77(1-2):68-79, 01 Sep 2002
Cited by: 42 articles | PMID: 12359132
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Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
Roe CR, Sweetman L, Roe DS, David F, Brunengraber H
J Clin Invest, 110(2):259-269, 01 Jul 2002
Cited by: 151 articles | PMID: 12122118 | PMCID: PMC151060
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Clinical effects of L-carnitine supplementation on apnea and growth in very low birth weight infants.
Whitfield J, Smith T, Sollohub H, Sweetman L, Roe CR
Pediatrics, 111(3):477-482, 01 Mar 2003
Cited by: 15 articles | PMID: 12612224
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