Acylcarnitines are fatty acid metabolites that play important roles in many cellular energy metabolism pathways. They have historically been used as important diagnostic markers for inborn errors of fatty acid oxidation and are being intensively studied as markers of energy metabolism, deficits in mitochondrial and peroxisomal β-oxidation activity, insulin resistance, and physical activity. Acylcarnitines are increasingly being identified as important indicators in metabolic studies of many diseases, including metabolic disorders, cardiovascular diseases, diabetes, depression, neurologic disorders, and certain cancers. The US Food and Drug Administration-approved drug L-carnitine, along with short-chain acylcarnitines (acetylcarnitine and propionylcarnitine), is now widely used as a dietary supplement. In light of their growing importance, we have undertaken an extensive review of acylcarnitines and provided a detailed description of their identity, nomenclature, classification, biochemistry, pathophysiology, supplementary use, potential drug targets, and clinical trials. We also summarize these updates in the Human Metabolome Database, which now includes information on the structures, chemical formulae, chemical/spectral properties, descriptions, and pathways for 1240 acylcarnitines. This work lays a solid foundation for identifying, characterizing, and understanding acylcarnitines in human biosamples. We also discuss the emerging opportunities for using acylcarnitines as biomarkers and as dietary interventions or supplements for many wide-ranging indications. The opportunity to identify new drug targets involved in controlling acylcarnitine levels is also discussed.

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酰基肉碱：命名法、生物标志物、治疗潜力、药物靶点和临床试验

酰基肉碱是脂肪酸代谢物，在许多细胞能量代谢途径中发挥重要作用。它们历来被用作先天性脂肪酸氧化错误的重要诊断标志物，并且作为能量代谢、线粒体和过氧化物酶体β缺陷的标志物正在被深入研究-氧化活性、胰岛素抵抗和体力活动。酰基肉碱越来越多地被确定为许多疾病代谢研究的重要指标，包括代谢紊乱、心血管疾病、糖尿病、抑郁症、神经系统疾病和某些癌症。美国食品和药物管理局批准的药物左旋肉碱，连同短链酰基肉碱（乙酰肉碱和丙酰肉碱），现在被广泛用作膳食补充剂。鉴于它们日益重要，我们对酰基肉碱进行了广泛的审查，并详细描述了它们的特性、命名、分类、生物化学、病理生理学、补充使用、潜在的药物靶点和临床试验。我们还在人类代谢组数据库中总结了这些更新，现在包括有关 1240 酰基肉碱的结构、化学式、化学/光谱特性、描述和途径的信息。这项工作为识别、表征和理解人类生物样本中的酰基肉碱奠定了坚实的基础。我们还讨论了使用酰基肉碱作为生物标志物以及作为饮食干预或补充剂用于许多广泛适应症的新兴机会。还讨论了确定涉及控制酰基肉碱水平的新药物靶点的机会。我们还讨论了使用酰基肉碱作为生物标志物以及作为饮食干预或补充剂用于许多广泛适应症的新兴机会。还讨论了确定涉及控制酰基肉碱水平的新药物靶点的机会。我们还讨论了使用酰基肉碱作为生物标志物以及作为饮食干预或补充剂用于许多广泛适应症的新兴机会。还讨论了确定涉及控制酰基肉碱水平的新药物靶点的机会。