메뉴 건너뛰기




Volumn 95, Issue 11, 2017, Pages 2236-2243

Triheptanoin for the treatment of brain energy deficit: A 14-year experience

Author keywords

astrocytes; GLUT1; Huntington disease; Krebs cycle; magnetic resonance spectroscopy

Indexed keywords

TRIHEPTANOIN; GLUCOSE; TRIACYLGLYCEROL;

EID: 85022178731     PISSN: 03604012     EISSN: 10974547     Source Type: Journal    
DOI: 10.1002/jnr.24111     Document Type: Review
Times cited : (37)

References (62)
  • 6
    • 1542348209 scopus 로고    scopus 로고
    • The energetics of Huntington's disease
    • Browne SE, Beal MF. 2004. The energetics of Huntington's disease. Neurochem Res 29:531–546.
    • (2004) Neurochem Res , vol.29 , pp. 531-546
    • Browne, S.E.1    Beal, M.F.2
  • 7
    • 33749042331 scopus 로고    scopus 로고
    • Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
    • Cui L, Jeong H, Borovecki F, Parkhurst CN, Tanese N, Krainc D. 2006. Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 127:59–69.
    • (2006) Cell , vol.127 , pp. 59-69
    • Cui, L.1    Jeong, H.2    Borovecki, F.3    Parkhurst, C.N.4    Tanese, N.5    Krainc, D.6
  • 8
    • 0025819954 scopus 로고
    • Defective glucose transport across the blood-brain barrier as a cause of persistent hypoglycorrhachia, seizures, and developmental delay
    • De Vivo DC, Trifiletti RR, Jacobson RI, Ronen GM, Behmand RA, Harik SI. 1991. Defective glucose transport across the blood-brain barrier as a cause of persistent hypoglycorrhachia, seizures, and developmental delay. N Engl J Med 325:703–709.
    • (1991) N Engl J Med , vol.325 , pp. 703-709
    • De Vivo, D.C.1    Trifiletti, R.R.2    Jacobson, R.I.3    Ronen, G.M.4    Behmand, R.A.5    Harik, S.I.6
  • 9
    • 70350453589 scopus 로고    scopus 로고
    • Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver
    • Deng S, Zhang GF, Kasumov T, Roe CR, Brunengraber H. 2009. Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver. J Biol Chem 284:27799–27807.
    • (2009) J Biol Chem , vol.284 , pp. 27799-27807
    • Deng, S.1    Zhang, G.F.2    Kasumov, T.3    Roe, C.R.4    Brunengraber, H.5
  • 10
    • 85011649822 scopus 로고    scopus 로고
    • Lack of appropriate stoichiometry: strong evidence against an energetically important astrocyte-neuron lactate shuttle in brain
    • Dienel GA. 2017. Lack of appropriate stoichiometry: strong evidence against an energetically important astrocyte-neuron lactate shuttle in brain. J Neurosci Res 95:2103–2125.
    • (2017) J Neurosci Res , vol.95 , pp. 2103-2125
    • Dienel, G.A.1
  • 11
    • 0037707488 scopus 로고    scopus 로고
    • Energy contribution of octanoate to intact rat brain metabolism measured by 13C nuclear magnetic resonance spectroscopy
    • Ebert D, Haller RG, Walton ME. 2003. Energy contribution of octanoate to intact rat brain metabolism measured by 13C nuclear magnetic resonance spectroscopy. J Neurosci 23:5928.
    • (2003) J Neurosci , vol.23 , pp. 5928
    • Ebert, D.1    Haller, R.G.2    Walton, M.E.3
  • 12
    • 84904196534 scopus 로고    scopus 로고
    • Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease
    • Francis JS, Markov V, Leone P. 2014. Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease. J Inherit Metab Dis 37:369–381.
    • (2014) J Inherit Metab Dis , vol.37 , pp. 369-381
    • Francis, J.S.1    Markov, V.2    Leone, P.3
  • 18
    • 74949136235 scopus 로고    scopus 로고
    • 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. 2010. Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile. Am J Physiol Endocrinol Metab 298:E362–E371.
    • (2010) Am J Physiol Endocrinol Metab , vol.298 , pp. E362-E371
    • Gu, L.1    Zhang, G.F.2    Kombu, R.S.3    Allen, F.4    Kutz, G.5    Brewer, W.U.6    Roe, C.R.7    Brunengraber, H.8
  • 19
    • 84897115053 scopus 로고    scopus 로고
    • Triheptanoin partially restores levels of tricarboxylic acid cycle intermediates in the mouse pilocarpine model of epilepsy
    • Hadera MG, Smeland OB, McDonald TS, Tan KN, Sonnewald U, Borges K. 2014. Triheptanoin partially restores levels of tricarboxylic acid cycle intermediates in the mouse pilocarpine model of epilepsy. J Neurochem 129:107–119.
    • (2014) J Neurochem , vol.129 , pp. 107-119
    • Hadera, M.G.1    Smeland, O.B.2    McDonald, T.S.3    Tan, K.N.4    Sonnewald, U.5    Borges, K.6
  • 20
    • 8344272718 scopus 로고    scopus 로고
    • The astrocyte-neuron lactate shuttle: a challenge of a challenge
    • Hertz L. 2004. The astrocyte-neuron lactate shuttle: a challenge of a challenge. J Cereb Blood Flow Metab 24:1241–1248.
    • (2004) J Cereb Blood Flow Metab , vol.24 , pp. 1241-1248
    • Hertz, L.1
  • 21
    • 0027480960 scopus 로고
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
    • Huntington's Disease Collaborative Research Group. 1993. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72:971–983.
    • (1993) Cell , vol.72 , pp. 971-983
  • 23
    • 84864102232 scopus 로고    scopus 로고
    • GLUT1 deficiency syndrome in clinical practice
    • Klepper J. 2012. GLUT1 deficiency syndrome in clinical practice. Epilepsy Res 100:272–277.
    • (2012) Epilepsy Res , vol.100 , pp. 272-277
    • Klepper, J.1
  • 24
    • 84880852464 scopus 로고    scopus 로고
    • Glut1 deficiency syndrome and novel ketogenic diets
    • Klepper J, Leiendecker B. 2013. Glut1 deficiency syndrome and novel ketogenic diets. J Child Neurol 28:1045–1048.
    • (2013) J Child Neurol , vol.28 , pp. 1045-1048
    • Klepper, J.1    Leiendecker, B.2
  • 28
  • 29
    • 24144463983 scopus 로고    scopus 로고
    • Metabolic control through the PGC-1 family of transcription coactivators
    • Lin J, Handschin C, Spiegelman BM. 2005. Metabolic control through the PGC-1 family of transcription coactivators. Cell Metab 1:361–370.
    • (2005) Cell Metab , vol.1 , pp. 361-370
    • Lin, J.1    Handschin, C.2    Spiegelman, B.M.3
  • 32
    • 84873411637 scopus 로고    scopus 로고
    • Heptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brain
    • Marin-Valencia I, Good LB, Ma Q, Malloy CR, Pascual JM. 2013. Heptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brain. J Cereb Blood Flow Metab 33:175–182.
    • (2013) J Cereb Blood Flow Metab , vol.33 , pp. 175-182
    • Marin-Valencia, I.1    Good, L.B.2    Ma, Q.3    Malloy, C.R.4    Pascual, J.M.5
  • 33
    • 24744444740 scopus 로고    scopus 로고
    • Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin
    • Milakovic T, Johnson GV. 2005. Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J Biol Chem 280:30773–30782.
    • (2005) J Biol Chem , vol.280 , pp. 30773-30782
    • Milakovic, T.1    Johnson, G.V.2
  • 40
    • 0030001887 scopus 로고    scopus 로고
    • Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease
    • Palfi S, Ferrante RJ, Brouillet E, Beal MF, Dolan R, Guyot MC, Peschanski M, Hantraye P. 1996. Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease. J Neurosci 16:3019–3025.
    • (1996) J Neurosci , vol.16 , pp. 3019-3025
    • Palfi, S.1    Ferrante, R.J.2    Brouillet, E.3    Beal, M.F.4    Dolan, R.5    Guyot, M.C.6    Peschanski, M.7    Hantraye, P.8
  • 41
    • 84907845373 scopus 로고    scopus 로고
    • 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. 2014. Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice. PLoS One 9:e109527.
    • (2014) PLoS One , vol.9
    • Park, M.J.1    Aja, S.2    Li, Q.3    Degano, A.L.4    Penati, J.5    Zhuo, J.6    Roe, C.R.7    Ronnett, G.V.8
  • 42
    • 84908007846 scopus 로고    scopus 로고
    • 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, et al. 2014. Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplement. JAMA Neurol 71:1255–1265.
    • (2014) JAMA Neurol , vol.71 , pp. 1255-1265
    • Pascual, J.M.1    Liu, P.2    Mao, D.3    Kelly, D.I.4    Hernandez, A.5    Sheng, M.6    Good, L.B.7    Ma, Q.8    Marin-Valencia, I.9    Zhang, X.10
  • 44
    • 0014683864 scopus 로고
    • Plasma-aminoacid levels in Huntington's chorea
    • Perry TL, Diamond S, Hansen S, Stedman D. 1969. Plasma-aminoacid levels in Huntington's chorea. Lancet 1:806–808.
    • (1969) Lancet , vol.1 , pp. 806-808
    • Perry, T.L.1    Diamond, S.2    Hansen, S.3    Stedman, D.4
  • 46
    • 77957241852 scopus 로고    scopus 로고
    • 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. 2010. Adult polyglucosan body disease (APBD): anaplerotic diet therapy (triheptanoin) and demonstration of defective methylation pathways. Mol Genet Metab 101:246–252.
    • (2010) Mol Genet Metab , vol.101 , pp. 246-252
    • Roe, C.R.1    Bottiglieri, T.2    Wallace, M.3    Arning, E.4    Martin, A.5
  • 47
    • 33745088995 scopus 로고    scopus 로고
    • Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential
    • Roe CR, Mochel F. 2006. Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential. J Inherit Metab Dis 29:332–340.
    • (2006) J Inherit Metab Dis , vol.29 , pp. 332-340
    • Roe, C.R.1    Mochel, F.2
  • 48
    • 0036071008 scopus 로고    scopus 로고
    • 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. 2002. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride. J Clin Invest 110:259–269.
    • (2002) J Clin Invest , vol.110 , pp. 259-269
    • Roe, C.R.1    Sweetman, L.2    Roe, D.S.3    David, F.4    Brunengraber, H.5
  • 50
    • 0026643890 scopus 로고
    • Effect of photic stimulation on human visual cortex lactate and phosphates using 1H and 31P magnetic resonance spectroscopy
    • Sappey-Marinier D, Calabrese G, Fein G, Hugg JW, Biggins C, Weiner MW. 1992. Effect of photic stimulation on human visual cortex lactate and phosphates using 1H and 31P magnetic resonance spectroscopy. J Cereb Blood Flow Metab 12:584–592.
    • (1992) J Cereb Blood Flow Metab , vol.12 , pp. 584-592
    • Sappey-Marinier, D.1    Calabrese, G.2    Fein, G.3    Hugg, J.W.4    Biggins, C.5    Weiner, M.W.6
  • 51
    • 0021988603 scopus 로고
    • Pyruvate carboxylase: an astrocyte-specific enzyme implicated in the replenishment of amino acid neurotransmitter pools
    • Shank RP, Bennett GS, Freytag SO, Campbell GL. 1985. Pyruvate carboxylase: an astrocyte-specific enzyme implicated in the replenishment of amino acid neurotransmitter pools. Brain Res 329:364–367.
    • (1985) Brain Res , vol.329 , pp. 364-367
    • Shank, R.P.1    Bennett, G.S.2    Freytag, S.O.3    Campbell, G.L.4
  • 54
    • 84879032355 scopus 로고    scopus 로고
    • Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data
    • Tabrizi SJ, Scahill RI, Owen G, Durr A, Leavitt BR, Roos RA, Borowsky B, Landwehrmeyer B, Frost C, Johnson H, et al.; TRACK-HD Investigators. 2013. Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data. Lancet Neurol 12:637–649.
    • (2013) Lancet Neurol , vol.12 , pp. 637-649
    • Tabrizi, S.J.1    Scahill, R.I.2    Owen, G.3    Durr, A.4    Leavitt, B.R.5    Roos, R.A.6    Borowsky, B.7    Landwehrmeyer, B.8    Frost, C.9    Johnson, H.10
  • 57
    • 84940894295 scopus 로고    scopus 로고
    • Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment—a retrospective chart review
    • Vockley J, Marsden D, McCracken E, DeWard S, Barone A, Hsu K, Kakkis E. 2015. Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment—a retrospective chart review. Mol Genet Metab 116:53–60.
    • (2015) Mol Genet Metab , vol.116 , pp. 53-60
    • Vockley, J.1    Marsden, D.2    McCracken, E.3    DeWard, S.4    Barone, A.5    Hsu, K.6    Kakkis, E.7
  • 59
    • 0022446533 scopus 로고
    • Abnormal skeletal muscle bioenergetics during exercise in patients with heart failure: role of reduced muscle blood flow
    • Wiener DH, Fink LI, Maris J, Jones RA, Chance B, Wilson JR. 1986. Abnormal skeletal muscle bioenergetics during exercise in patients with heart failure: role of reduced muscle blood flow. Circulation 73:1127–1136.
    • (1986) Circulation , vol.73 , pp. 1127-1136
    • Wiener, D.H.1    Fink, L.I.2    Maris, J.3    Jones, R.A.4    Chance, B.5    Wilson, J.R.6
  • 60
    • 77957790307 scopus 로고    scopus 로고
    • Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models
    • Willis S, Stoll J, Sweetman L, Borges K. 2010. Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models. Neurobiol Dis 40:565–572.
    • (2010) Neurobiol Dis , vol.40 , pp. 565-572
    • Willis, S.1    Stoll, J.2    Sweetman, L.3    Borges, K.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.