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. 2021 Nov 4;11(11):1633. doi: 10.3390/biom11111633

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© 2021 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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Schematic representation of altered mitochondrial enzymes in NMDs. Mitochondrial metabolic pathways involved in NMDs are shown, and mutated enzymes are highlighted in red. For details, see the text. α-KG, α-ketoglutarate; ACAD, acyl-CoA dehydrogenase; ACO, aconitase; ASP, aspartate; CIT, citrate; CoASH, coenzyme A; ECH, 2-enoyl-CoA hydratase; FUM, fumarate; IDH, isocitrate dehydrogenase; ISO, isocitrate; MAL, malate; MDH, malate dehydrogenase; Mfn1/2, mitofusins 1 and 2; OAA, oxaloacetate; OPA1, optic atrophy 1 protein; SDH, succinate dehydrogenase; SUC, succinate; TCA, tricarboxylic acid cycle.