Triheptanoin (BioDeep_00000184528)
human metabolite blood metabolite
代谢物信息卡片
1,3-bis(heptanoyloxy)propan-2-yl heptanoate
化学式: C24H44O6 (428.3137724)
中文名称: 三庚酸甘油酯
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CCCCCCC(=O)OCC(COC(=O)CCCCCC)OC(=O)CCCCCC
InChI: InChI=1S/C24H44O6/c1-4-7-10-13-16-22(25)28-19-21(30-24(27)18-15-12-9-6-3)20-29-23(26)17-14-11-8-5-2/h21H,4-20H2,1-3H3
描述信息
A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AX - Various alimentary tract and metabolism products
同义名列表
数据库引用编号
7 个数据库交叉引用编号
- PubChem: 69286
- HMDB: HMDB0259217
- DrugBank: DB11677
- ChEMBL: CHEMBL4297585
- Wikipedia: Triheptanoin
- chemspider: 62497
- CAS: 620-67-7
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
1 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Erru Yang, Eliza Kruger, David Yin, Kieran Mace, Meghan Tierney, Noelle Liao, Emily Cibelli, Dan Drozd, Nathan Ross, Kathleen L Deering, Peter Herout, Qing Harshaw, Alicia Shillington, Nina Thomas, Deborah Marsden, Amy Kritzer, Jerry Vockley. Major clinical events and healthcare resource use among patients with long-chain fatty acid oxidation disorders in the United States: Results from LC-FAOD Odyssey program.
Molecular genetics and metabolism.
2024 May; 142(1):108350. doi:
10.1016/j.ymgme.2024.108350. [PMID: 38458123] - Jerry Vockley, Barbara K Burton, Gerard Berry, Nicola Longo, John Phillips, Amarilis Sanchez-Valle, Kimberly A Chapman, Pranoot Tanpaiboon, Stephanie Grunewald, Elaine Murphy, Xiaoxiao Lu, Syeda Rahman, Kathryn Ray, Bridget Reineking, Laura Pisani, Antonio Nino Ramirez. Triheptanoin for the treatment of long-chain fatty acid oxidation disorders: Final results of an open-label, long-term extension study.
Journal of inherited metabolic disease.
2023 09; 46(5):943-955. doi:
10.1002/jimd.12640. [PMID: 37276053] - Ayca Burcu Kahraman, Yılmaz Yildiz, Hulya Gokmen-Ozel, Sibel Kadayifcilar, Serap Sivri. Successful management of rhabdomyolysis with triheptanoin in a child with severe long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency.
Neuromuscular disorders : NMD.
2023 Apr; 33(4):315-318. doi:
10.1016/j.nmd.2023.02.008. [PMID: 36893607] - Nelida Inés Noguera, Daniela Tavian, Corrado Angelini, Francesca Cortese, Massimiliano Filosto, Matteo Garibaldi, Sara Missaglia, Ariela Smigliani, Alessandra Zaza, Elena Maria Pennisi. Effects of Triheptanoin on Mitochondrial Respiration and Glycolysis in Cultured Fibroblasts from Neutral Lipid Storage Disease Type M (NLSD-M) Patients.
Biomolecules.
2023 03; 13(3):. doi:
10.3390/biom13030452. [PMID: 36979387] - Sun Ku Lee, Nathalie H Gosselin, Claudia Jomphe, Kathleen McKeever, Wendy Putnam. Population Pharmacokinetics of Heptanoate in Healthy Subjects and Patients With Long-Chain Fatty Acid Oxidation Disorders Treated With Triheptanoin.
Clinical pharmacology in drug development.
2022 11; 11(11):1264-1272. doi:
10.1002/cpdd.1145. [PMID: 35908210] - Jerry Vockley, Gregory M Enns, Antonio Nino Ramirez, Camille L Bedrosian, Bridget Reineking, Xiaoxiao Lu, Kathryn Ray, Syeda Rahman, Deborah Marsden. Response to triheptanoin therapy in critically ill patients with LC-FAOD: Report of patients treated through an expanded access program.
Molecular genetics and metabolism.
2022 06; 136(2):152-162. doi:
10.1016/j.ymgme.2022.04.001. [PMID: 35459555] - Sun Ku Lee, Manju Gupta, Jack Shi, Kathleen McKeever. The Pharmacokinetics of Triheptanoin and Its Metabolites in Healthy Subjects and Patients With Long-Chain Fatty Acid Oxidation Disorders.
Clinical pharmacology in drug development.
2021 11; 10(11):1325-1334. doi:
10.1002/cpdd.944. [PMID: 33789001] - Kristin Engelstad, Rachel Salazar, Dorcas Koenigsberger, Erin Stackowtiz, Susan Brodlie, Melanie Brandabur, Darryl C De Vivo. Exploring triheptanoin as treatment for short chain enoyl CoA hydratase deficiency.
Annals of clinical and translational neurology.
2021 05; 8(5):1151-1157. doi:
10.1002/acn3.51359. [PMID: 33931985] - Dina Zand, Jenny Doan, Sojeong Yi, Jie Wang, Lian Ma, Emmanuel Akinshola, Sushanta Chakder, Joette Meyer, Michael Pacanowski, Laura Lee Johnson, Kathleen Donohue, Julie Beitz. Regulatory news: Dojolvi (triheptanoin) as a source of calories and fatty acids in long-chain fatty acid oxidation disorders: FDA approval summary.
Journal of inherited metabolic disease.
2021 05; 44(3):515-517. doi:
10.1002/jimd.12377. [PMID: 33729583] - Marie K Norris, Anna I Scott, Sarah Sullivan, Irene J Chang, Christina Lam, Angela Sun, Sihoun Hahn, Jenny M Thies, Melissa Gunnarson, Kelly N McKean, J Lawrence Merritt. Tutorial: Triheptanoin and Nutrition Management for Treatment of Long-Chain Fatty Acid Oxidation Disorders.
JPEN. Journal of parenteral and enteral nutrition.
2021 02; 45(2):230-238. doi:
10.1002/jpen.2034. [PMID: 33085788] - Jerry Vockley, Nicola Longo, Megan Madden, Lauren Dwyer, Yunming Mu, Chao-Yin Chen, Jason Cataldo. Dietary management and major clinical events in patients with long-chain fatty acid oxidation disorders enrolled in a phase 2 triheptanoin study.
Clinical nutrition ESPEN.
2021 02; 41(?):293-298. doi:
10.1016/j.clnesp.2020.11.018. [PMID: 33487279] - Thomas Zöggeler, Katharina Stock, Monika Jörg-Streller, Johannes Spenger, Vassiliki Konstantopoulou, Miriam Hufgard-Leitner, Sabine Scholl-Bürgi, Daniela Karall. Long-term experience with triheptanoin in 12 Austrian patients with long-chain fatty acid oxidation disorders.
Orphanet journal of rare diseases.
2021 01; 16(1):28. doi:
10.1186/s13023-020-01635-x. [PMID: 33446227] - Jerry Vockley, Barbara Burton, Gerard Berry, Nicola Longo, John Phillips, Amarilis Sanchez-Valle, Kimberly Chapman, Pranoot Tanpaiboon, Stephanie Grunewald, Elaine Murphy, Xiaoxiao Lu, Jason Cataldo. Effects of triheptanoin (UX007) in patients with long-chain fatty acid oxidation disorders: Results from an open-label, long-term extension study.
Journal of inherited metabolic disease.
2021 01; 44(1):253-263. doi:
10.1002/jimd.12313. [PMID: 32885845] - Garen Gaston, Jon A Gangoiti, Shelley Winn, Benjamin Chan, Bruce A Barshop, Cary O Harding, Melanie B Gillingham. Cardiac tissue citric acid cycle intermediates in exercised very long-chain acyl-CoA dehydrogenase-deficient mice fed triheptanoin or medium-chain triglyceride.
Journal of inherited metabolic disease.
2020 11; 43(6):1232-1242. doi:
10.1002/jimd.12284. [PMID: 33448436] - Matt Shirley. Triheptanoin: First Approval.
Drugs.
2020 Oct; 80(15):1595-1600. doi:
10.1007/s40265-020-01399-5. [PMID: 32897506] - Ramona De Amicis, Alessandro Leone, Stefano Ravasenghi, Graziana Scigliuolo, Elena Mauro, Ettore Salsano, Alberto Battezzati, Simona Bertoli. Triheptanoin Supplementation Does not Affect Nutritional Status: A Case Report of Two Siblings With Adult Polyglucosan Body Disease.
Journal of the American College of Nutrition.
2020 08; 39(6):557-562. doi:
10.1080/07315724.2019.1695233. [PMID: 31860384] - Tanya McDonald, Mark P Hodson, Ilya Bederman, Michelle Puchowicz, Karin Borges. 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.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
2020 03; 40(3):678-691. doi:
10.1177/0271678x19837380. [PMID: 30890077] - Karen L Madsen, Pascal Laforêt, Astrid E Buch, Mads G Stemmerik, Chris Ottolenghi, Stéphane N Hatem, Daniel T Raaschou-Pedersen, Nanna S Poulsen, Maria Atencio, Marie-Pierre Luton, Alexandre Ceccaldi, Ronald G Haller, Ros Quinlivan, Fanny Mochel, John Vissing. No effect of triheptanoin on exercise performance in McArdle disease.
Annals of clinical and translational neurology.
2019 10; 6(10):1949-1960. doi:
10.1002/acn3.50863. [PMID: 31520525] - John M Shoffner. Concerning 'Triheptanoin vs trioctanoin for long-chain fatty acid oxidation disorders: A double blinded, randomized controlled trial' by Gillingham et al.
Journal of inherited metabolic disease.
2019 05; 42(3):394-395. doi:
10.1002/jimd.12078. [PMID: 30838661] - Keri J Fogle, Amber R Smith, Sidney L Satterfield, Alejandra C Gutierrez, J Ian Hertzler, Caleb S McCardell, Joy H Shon, Zackery J Barile, Molly O Novak, Michael J Palladino. Ketogenic and anaplerotic dietary modifications ameliorate seizure activity in Drosophila models of mitochondrial encephalomyopathy and glycolytic enzymopathy.
Molecular genetics and metabolism.
2019 04; 126(4):439-447. doi:
10.1016/j.ymgme.2019.01.008. [PMID: 30683556] - Kah Ni Tan, David Simmons, Catalina Carrasco-Pozo, Karin Borges. Triheptanoin protects against status epilepticus-induced hippocampal mitochondrial dysfunctions, oxidative stress and neuronal degeneration.
Journal of neurochemistry.
2018 02; 144(4):431-442. doi:
10.1111/jnc.14275. [PMID: 29222946] - Melanie B Gillingham, Stephen B Heitner, Julie Martin, Sarah Rose, Amy Goldstein, Areeg Hassan El-Gharbawy, Stephanie Deward, Michael R Lasarev, Jim Pollaro, James P DeLany, Luke J Burchill, Bret Goodpaster, James Shoemaker, Dietrich Matern, Cary O Harding, Jerry Vockley. Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Journal of inherited metabolic disease.
2017 11; 40(6):831-843. doi:
10.1007/s10545-017-0085-8. [PMID: 28871440] - Konrad Koch, Dirk Berressem, Jan Konietzka, Anna Thinnes, Gunter P Eckert, Jochen Klein. Hepatic Ketogenesis Induced by Middle Cerebral Artery Occlusion in Mice.
Journal of the American Heart Association.
2017 Apr; 6(4):. doi:
10.1161/jaha.117.005556. [PMID: 28381467] - J Vockley, B Burton, G T Berry, N Longo, J Phillips, A Sanchez-Valle, P Tanpaiboon, S Grunewald, E Murphy, R Humphrey, J Mayhew, A Bowden, L Zhang, J Cataldo, D L Marsden, E Kakkis. UX007 for the treatment of long chain-fatty acid oxidation disorders: Safety and efficacy in children and adults following 24weeks of treatment.
Molecular genetics and metabolism.
2017 04; 120(4):370-377. doi:
10.1016/j.ymgme.2017.02.005. [PMID: 28189603] - Tine M Comhair, Sonia C Garcia Caraballo, Cornelis H C Dejong, Wouter H Lamers, S Eleonore Koehler. The odd-carbon medium-chain fatty triglyceride triheptanoin does not reduce hepatic steatosis.
Clinical nutrition (Edinburgh, Scotland).
2017 02; 36(1):229-237. doi:
10.1016/j.clnu.2015.11.005. [PMID: 26778339] - Sara Tucci, Ulrich Floegel, Frauke Beermann, Sidney Behringer, Ute Spiekerkoetter. Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase-deficient mouse.
Journal of lipid research.
2017 01; 58(1):196-207. doi:
10.1194/jlr.m072033. [PMID: 27884962] - J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden. Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders.
Molecular genetics and metabolism.
2016 11; 119(3):223-231. doi:
10.1016/j.ymgme.2016.08.008. [PMID: 27590926] - Charles R Roe, Henri Brunengraber. Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.
Molecular genetics and metabolism.
2015 Dec; 116(4):260-8. doi:
10.1016/j.ymgme.2015.10.005. [PMID: 26547562] - Sara Tucci, Sidney Behringer, Ute Spiekerkoetter. 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.
The FEBS journal.
2015 Nov; 282(21):4242-53. doi:
10.1111/febs.13418. [PMID: 26284828] - Jerry Vockley, Deborah Marsden, Elizabeth McCracken, Stephanie DeWard, Amanda Barone, Kristen Hsu, Emil Kakkis. 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.
Molecular genetics and metabolism.
2015 Sep; 116(1-2):53-60. doi:
10.1016/j.ymgme.2015.06.006. [PMID: 26116311] - T M Schwarzkopf, K Koch, J Klein. Reduced severity of ischemic stroke and improvement of mitochondrial function after dietary treatment with the anaplerotic substance triheptanoin.
Neuroscience.
2015 Aug; 300(?):201-9. doi:
10.1016/j.neuroscience.2015.05.014. [PMID: 25982559] - Ingrid Sofia Vieira de Melo, Terezinha Da Rocha Ataide, Suzana Lima de Oliveira, Nassib Bezerra Bueno, Johnnatan Duarte de Freitas, Antônio Euzébio Goulart Sant'Ana. HEPATIC FATTY ACID PROFILE OF RATS FED A TRIHEPTANOIN-BASED KETOGENIC DIET.
Nutricion hospitalaria.
2015 Jul; 32(1):265-9. doi:
10.3305/nh.2015.32.1.9033. [PMID: 26262726] - Iclea Rocha Gama, Euclides Marinho Trindade-Filho, Suzana Lima Oliveira, Nassib Bezerra Bueno, Isabelle Tenório Melo, Cyro Rego Cabral-Junior, Elenita M Barros, Jaqueline A Galvão, Wanessa S Pereira, Raphaela C Ferreira, Bruna R Domingos, Terezinha da Rocha Ataide. Effects of ketogenic diets on the occurrence of pilocarpine-induced status epilepticus of rats.
Metabolic brain disease.
2015 Feb; 30(1):93-8. doi:
10.1007/s11011-014-9586-4. [PMID: 25005004] - C Breen, F J White, C A B Scott, L Heptinstall, J H Walter, S A Jones, A A M Morris. Unsuccessful treatment of severe pyruvate carboxylase deficiency with triheptanoin.
European journal of pediatrics.
2014 Mar; 173(3):361-6. doi:
10.1007/s00431-013-2166-5. [PMID: 24114256] - Min Jung Park, Susan Aja, Qun Li, Alicia L Degano, Judith Penati, Justin Zhuo, Charles R Roe, Gabriele V Ronnett. Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice.
PloS one.
2014; 9(10):e109527. doi:
10.1371/journal.pone.0109527. [PMID: 25299635] - Isaac Marin-Valencia, Levi B Good, Qian Ma, Craig R Malloy, Juan M Pascual. Heptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brain.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.
2013 Feb; 33(2):175-82. doi:
10.1038/jcbfm.2012.151. [PMID: 23072752] - C Ramachandran, K-W Quirin, A Resek, S J Melnick. Protective and restorative effects of a Commiphora mukul gum resin and triheptanoin preparation on the CCL-110 skin fibroblast cell line.
International journal of cosmetic science.
2012 Apr; 34(2):155-60. doi:
10.1111/j.1468-2494.2011.00695.x. [PMID: 22084831] - Charles R Roe, Teodoro Bottiglieri, Mary Wallace, Erland Arning, Alan Martin. Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways.
Molecular genetics and metabolism.
2010 Oct; 101(2-3):246-52. doi:
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Journal of inherited metabolic disease.
2010 Oct; 33(5):555-61. doi:
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European journal of human genetics : EJHG.
2010 Sep; 18(9):1057-60. doi:
10.1038/ejhg.2010.72. [PMID: 20512158] - Lisa A Borgia, Stephanie J Valberg, Molly E McCue, Joe D Pagan, Charles R Roe. 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.
American journal of veterinary research.
2010 Mar; 71(3):326-36. doi:
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Journal of biomedicine & biotechnology.
2010; 2010(?):340849. doi:
10.1155/2010/340849. [PMID: 20589068] - Molly E McCue, Stephanie J Valberg, Joe D Pagan, Birgitta Essén-Gustavsson, Charles R Roe. Effect of triheptanoin on muscle metabolism during submaximal exercise in horses.
American journal of veterinary research.
2009 Aug; 70(8):1043-52. doi:
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Arquivos brasileiros de endocrinologia e metabologia.
2008 Oct; 52(7):1145-55. doi:
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Neuroscience letters.
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American journal of physiology. Endocrinology and metabolism.
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Journal of inherited metabolic disease.
2006 Apr; 29(2-3):332-40. doi:
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