Figure 1. Main stages in carnitine and fatty acid ß-oxidation cycle. Uptake and activation of FA, in gray: FATP, FAT/CD36: fatty acid transporters; AS: acyl-CoA synthetase; Cycling of carnitine to pass the FA to the mitochondrial matrix, in red: OCTN2: carnitine transporter; CPT I: carnitine palmitoyltransferase I; CACT: carnitine-acylcarnitine translocase; CPT II: carnitine palmitoyltransferase II; β-Oxidation spiral, in green: SCAD: short-chain acyl-CoA dehydrogenase; MCAD: medium-chain acyl-CoA dehydrogenase; VLCAD: very long-chain acyl-CoA dehydrogenase; SCEH: short-chain enoyl-CoA hydratase; LCEH: long-chain enoyl-CoA hydratase; HAD (M/SCHAD): 3-hydroxyacyl-CoA dehydrogenase; LCHAD: long-chain 3-hydroxyacyl-CoA dehydrogenase; S/MCKAT: short/medium-chain 3-ketoacyl-CoA thiolase; LCKAT: long-chain 3-ketoacyl-CoA thiolase; MTP: mitochondrial trifunctional protein; MAD: multiple acyl-CoA dehydrogenase; Electron transfer and respiratory chain pathway, in blue: CoQ, CI, CII, CIII, CIV, CV: coenzyme Q and mitochondrial respiratory complexes I, II, III, IV and V. [13]

From

A Case Study of Very Long Fatty Acid Dehydrogenase Deficiency Presented by Irritability and Seizures

S. AlKaabi, R. Ebead, M. Khames, M. Amin, S. Ahmed, A. Gupta, E. Marzouk

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