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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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Current therapeutic options targeting mitochondrial dysfunction in PAH. Acyl-CoA: acyl-coenzyme A; DON: 6-Diazo-5-oxo-L-norleucine; DRP-1: dynamin-related protein 1; FAO: fatty acid oxidation; GLUT 1: glucose transporter 1; G6P: glucose-6-phosphate; HIF: hypoxia inducible factor; HK: hexokinase; HRE: hypoxia response element; LDHA: lactate dehydrogenase; Mdivi-1: mitochondrial division inhibitor 1; MFN-2: mitofusin-1; MPT: mitochondrial pyruvate transporter; mTOr: mammalian target of rapamycin; NFAT: Nuclear factor of activated T-cells; 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.