Therapeutic potential of triheptanoin in metabolic and neurodegenerative diseases
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[1] M. Hodson,et al. 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 , 2020, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
[2] P. Laforêt,et al. No effect of triheptanoin on exercise performance in McArdle disease , 2019, Annals of clinical and translational neurology.
[3] L. Rocha,et al. Drug-resistant epilepsy: From multiple hypotheses to an integral explanation using preclinical resources , 2019, Epilepsy & Behavior.
[4] K. J. Fogle,et al. Ketogenic and anaplerotic dietary modifications ameliorate seizure activity in Drosophila models of mitochondrial encephalomyopathy and glycolytic enzymopathy. , 2019, Molecular genetics and metabolism.
[5] P. Kwan,et al. Randomized trial of add‐on triheptanoin vs medium chain triglycerides in adults with refractory epilepsy , 2019, Epilepsia open.
[6] R. Schiffmann,et al. A double-blind, placebo-controlled trial of triheptanoin in adult polyglucosan body disease and open-label, long-term outcome , 2018, Journal of Inherited Metabolic Disease.
[7] K. Borges,et al. Impairments in Oxidative Glucose Metabolism in Epilepsy and Metabolic Treatments Thereof , 2018, Front. Cell. Neurosci..
[8] K. Borges,et al. A pilot study of add-on oral triheptanoin treatment for children with medically refractory epilepsy. , 2018, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.
[9] K. Borges,et al. Heptanoate is neuroprotective in vitro but triheptanoin post-treatment did not protect against middle cerebral artery occlusion in rats , 2018, Neuroscience Letters.
[10] I. Scheffer,et al. Epilepsy , 2018, Nature Reviews Disease Primers.
[11] K. Borges,et al. Triheptanoin protects against status epilepticus‐induced hippocampal mitochondrial dysfunctions, oxidative stress and neuronal degeneration , 2018, Journal of neurochemistry.
[12] J. Kirschner,et al. Mitochondrial fatty acid biosynthesis and muscle fiber plasticity in very long‐chain acyl‐CoA dehydrogenase‐deficient mice , 2018, FEBS letters.
[13] M. Vidailhet,et al. A randomized, controlled, double-blind, crossover trial of triheptanoin in alternating hemiplegia of childhood , 2017, Orphanet Journal of Rare Diseases.
[14] M. Iruela-Arispe,et al. Membrane lipids and cell signaling , 2017, Current opinion in lipidology.
[15] J. Vissing,et al. Treatment Opportunities in Patients With Metabolic Myopathies , 2017, Current Treatment Options in Neurology.
[16] J. DeLany,et al. Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial , 2017, Journal of Inherited Metabolic Disease.
[17] F. Mochel. Triheptanoin for the treatment of brain energy deficit: A 14‐year experience , 2017, Journal of neuroscience research.
[18] H. Lerche,et al. The role of genetic testing in epilepsy diagnosis and management , 2017, Expert review of molecular diagnostics.
[19] P. Escribá,et al. Triacylglycerol mimetics regulate membrane interactions of glycogen branching enzyme: implications for therapy[S] , 2017, Journal of Lipid Research.
[20] K. Borges,et al. Alternative Fuels in Epilepsy and Amyotrophic Lateral Sclerosis , 2017, Neurochemical Research.
[21] J. Vockley,et al. UX007 for the treatment of long chain-fatty acid oxidation disorders: Safety and efficacy in children and adults following 24weeks of treatment. , 2017, Molecular genetics and metabolism.
[22] S. Tucci. Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency–studies on treatment effects and long-term outcomes in mouse models , 2017, Journal of Inherited Metabolic Disease.
[23] C. Dejong,et al. The odd-carbon medium-chain fatty triglyceride triheptanoin does not reduce hepatic steatosis. , 2017, Clinical nutrition.
[24] S. Tucci,et al. Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase-deficient mouse[S] , 2016, Journal of Lipid Research.
[25] J. Charrow,et al. Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders. , 2016, Molecular genetics and metabolism.
[26] K. Borges,et al. Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral Sclerosis , 2016, PloS one.
[27] K. Borges,et al. Modification of Astrocyte Metabolism as an Approach to the Treatment of Epilepsy: Triheptanoin and Acetyl-l-Carnitine , 2016, Neurochemical Research.
[28] C. Roe,et al. Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience. , 2015, Molecular genetics and metabolism.
[29] R. Schiffmann,et al. Triheptanoin dramatically reduces paroxysmal motor disorder in patients with GLUT1 deficiency , 2015, Journal of Neurology, Neurosurgery & Psychiatry.
[30] S. Tucci,et al. De novo fatty acid biosynthesis and elongation in very long‐chain acyl‐CoA dehydrogenase‐deficient mice supplemented with odd or even medium‐chain fatty acids , 2015, The FEBS journal.
[31] J. Klein,et al. Reduced severity of ischemic stroke and improvement of mitochondrial function after dietary treatment with the anaplerotic substance triheptanoin , 2015, Neuroscience.
[32] Romain Valabregue,et al. Triheptanoin improves brain energy metabolism in patients with Huntington disease , 2015, Neurology.
[33] A. Sherry,et al. The ratio of acetate‐to‐glucose oxidation in astrocytes from a single 13C NMR spectrum of cerebral cortex , 2015, Journal of neurochemistry.
[34] Min Jung Park,et al. Anaplerotic Triheptanoin Diet Enhances Mitochondrial Substrate Use to Remodel the Metabolome and Improve Lifespan, Motor Function, and Sociability in MeCP2-Null Mice , 2014, PloS one.
[35] Jason Y. Park,et al. Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplement. , 2014, JAMA neurology.
[36] P. Leone,et al. Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease , 2014, Journal of Inherited Metabolic Disease.
[37] J. Walter,et al. Unsuccessful treatment of severe pyruvate carboxylase deficiency with triheptanoin , 2014, European Journal of Pediatrics.
[38] M. Vidailhet,et al. GLUT1 deficiency syndrome: an update. , 2014, Revue neurologique.
[39] M. Hodson,et al. Alterations of Hippocampal Glucose Metabolism by Even versus Uneven Medium Chain Triglycerides , 2014, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
[40] P. Rijken,et al. Checks and balances in membrane phospholipid class and acyl chain homeostasis, the yeast perspective. , 2013, Progress in lipid research.
[41] I. Ferrer,et al. Triheptanoin supplementation to ketogenic diet curbs cognitive impairment in APP/PS1 mice used as a model of familial Alzheimer's disease. , 2013, Current Alzheimer research.
[42] L. Good,et al. Heptanoate as a Neural Fuel: Energetic and Neurotransmitter Precursors in Normal and Glucose Transporter I-Deficient (G1D) Brain , 2013, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
[43] C. Reid,et al. Triheptanoin reduces seizure susceptibility in a syndrome-specific mouse model of generalized epilepsy , 2013, Epilepsy Research.
[44] L. Good,et al. Glut1 deficiency (G1D): Epilepsy and metabolic dysfunction in a mouse model of the most common human phenotype , 2012, Neurobiology of Disease.
[45] K. Borges,et al. Triheptanoin—A medium chain triglyceride with odd chain fatty acids: A new anaplerotic anticonvulsant treatment? , 2012, Epilepsy Research.
[46] J. Klepper. GLUT1 deficiency syndrome in clinical practice , 2012, Epilepsy Research.
[47] K. Borges,et al. Triheptanoin in acute mouse seizure models , 2012, Epilepsy Research.
[48] K. Borges,et al. Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models , 2010, Neurobiology of Disease.
[49] W. Blume. Focal and Generalized: Both Here and There , 2010, Epilepsy currents.
[50] A. Durr,et al. Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease , 2010, European Journal of Human Genetics.
[51] Guo-Fang Zhang,et al. Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats . II . Effects on lipolysis , glucose production , and liver acyl-CoA profile , 2010 .
[52] T. Bottiglieri,et al. Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways. , 2009, Molecular genetics and metabolism.
[53] A. Sant'ana,et al. [Hepatoprotective effect of diheptanoin and tritreptanoin chronic consumption against steatosis in rats]. , 2008, Arquivos brasileiros de endocrinologia e metabologia.
[54] C. Roe,et al. Carnitine palmitoyltransferase II deficiency , 2008, Neurology.
[55] D. Thurman,et al. How common are the “common” neurologic disorders? , 2007, Neurology.
[56] G. Kutz,et al. Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. , 2006, American journal of physiology. Endocrinology and metabolism.
[57] F. Mochel,et al. Anaplerotic diet therapy in inherited metabolic disease: Therapeutic potential , 2006, Journal of Inherited Metabolic Disease.
[58] C. Roe,et al. Anaplerotic molecules: Current and future , 2006, Journal of Inherited Metabolic Disease.
[59] F. Mochel,et al. Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy. , 2005, Molecular genetics and metabolism.
[60] C. Roe,et al. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride. , 2002, The Journal of clinical investigation.
[61] K. Alberti,et al. Pyruvate carboxylase deficiency , 1984, Journal of Inherited Metabolic Disease.
[62] L. Deenen,et al. Membrane asymmetry and blood coagulation , 1977, Nature.
[63] G. Enns,et al. Triheptanoin: A Rescue Therapy for Cardiogenic Shock in Carnitine-acylcarnitine Translocase Deficiency. , 2018, JIMD reports.
[64] P. Legrand,et al. Revisiting the metabolism and physiological functions of caprylic acid (C8:0) with special focus on ghrelin octanoylation. , 2016, Biochimie.