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. 2023 May 31;24(11):9572. doi: 10.3390/ijms24119572

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© 2023 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).

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Main mitochondrial metabolic pathways involved in PAH. Acyl-CoA: acyl-coenzyme A; CPT-1: carnitine palmitoyltransferase 1; DRP-1: dynamin-related protein 1; Fe–S: iron-sulfur; GLUT 1: glucose transporter 1; G6P: glucose-6-phosphate; HIF: hypoxia inducible factor; HK: hexokinase; HRE: hypoxia response element; LDHA: lactate dehydrogenase; MCD: malonyl-CoA decarboxylase; MFN-2: mitofusin-1; MPT: mitochondrial pyruvate transporter; PDH: pyruvate dehydrogenase, PDK: pyruvate dehydrogenase kinase; PEP: phosphoenolpyruvate; PGC 1: Peroxisome proliferator-activated receptor-gamma coactivator; PKM2: pyruvate kinase isoform M2; TCA cycle: tricarboxylic acid cycle.