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. 2021 Mar 2;11:153. doi: 10.1038/s41398-020-01097-6

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© The Author(s) 2021

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — Mitochondrial energy metabolism pathways involve branched-chain amino acids (BCAAs), acylcarnitines, fatty acid beta-oxidation, and tricarboxylic acid cycle (TCA cycle). A delicate balance between two of the main cellular energy resources, glucose utilization, and fatty acid utilization, is essential to maintain physiological homeostasis, the perturbation of which may lead to pathologic processes. The gray font metabolites have not been measured in p180. Leu leucine, Val valine, Ile isoleucine, CoA coenzyme A, CPT1 carnitine-palmitoyl-transferase 1, CPT2 carnitine-palmitoyl-transferase 2, CACT carnitine-acylcarnitine-translocase, CrAT carnitine O-acetyltransferase. Metabolite abbreviations are spelled out in Supplementary Table 1.