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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Long-term experience with triheptanoin in 12 Austrian patients with long-chain fatty acid oxidation disorders

Fig. 1

Diagram of the mitochondrial metabolic LC-FAOD and C7 pathways. Abbreviations: CPT1: carnitine palmitoyltransferase 1; CPT2: carnitine palmitoyltransferase 2; VLCAD: very long-chain acyl-CoA dehydrogenase; MCAD: medium-chain acyl-CoA dehydrogenase; SCAD: short-chain acyl-CoA dehydrogenase; LCHAD: long-chain 3-hydroxyacyl-CoA dehydrogenase; SCHAD: short-chain 3-hydroxylacyl-CoA dehydrogenase; LCKAT: long-chain 3-ketothiolase; MCKAT: medium-chain 3-ketoacyl-CoA thiolase; PROP-CoA: propionyl-CoA; FAO: fatty acid oxidation; CAC: citric acid cycle; ATP: adenosine triphosphate. Copyright Thomas Zöggeler MD

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172