Laboratory of Clinical Biochemistry and Metabolism, Department of General Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, Freiburg, Germany.
Faculty of Biology, University of Freiburg, Freiburg, Germany.
J Inherit Metab Dis. 2020 May;43(3):385-391. doi: 10.1002/jimd.12199. Epub 2019 Dec 12.
In the past 15 years the potential of triheptanoin for the treatment of several human diseases in the area of clinical nutrition has grown considerably. Use of this triglyceride of the odd-chain fatty acid heptanoate has been proposed and applied for the treatment of several conditions in which the energy supply from citric acid cycle intermediates or fatty acid degradation are impaired. Neurological diseases due to disturbed glucose metabolism or metabolic diseases associated with impaired β-oxidation of long chain fatty acid may especially take advantage of alternative substrate sources offered by the secondary metabolites of triheptanoin. Epilepsy due to deficiency of the GLUT1 transporter, as well as diseases associated with dysregulation of neuronal signalling, have been treated with triheptanoin supplementation, and very recently the advantages of this oil in long-chain fatty acid oxidation disorders have been reported. The present review summarises the published literature on the metabolism of triheptanoin including clinical reports related to the use of triheptanoin.
在过去的 15 年中，三庚酸甘油酯在临床营养领域治疗多种人类疾病的潜力显著增加。这种庚酸奇数链脂肪酸的三酸甘油酯已被提议并应用于治疗多种情况下的柠檬酸循环中间体或脂肪酸降解受损的能量供应。由于葡萄糖代谢紊乱引起的神经疾病或与长链脂肪酸β氧化受损相关的代谢疾病，可能特别受益于三庚酸甘油酯的次级代谢产物提供的替代底物来源。由于 GLUT1 转运蛋白缺乏引起的癫痫，以及与神经元信号调节紊乱相关的疾病，都已通过三庚酸甘油酯补充进行了治疗，最近还报道了这种油在长链脂肪酸氧化紊乱中的优势。本综述总结了关于三庚酸甘油酯代谢的已发表文献，包括与三庚酸甘油酯使用相关的临床报告。
