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Fatty acid oxidation metabolism overview

Fatty acid oxidation metabolism overview
Figure illustrating the carnitine cycle. The mitochondrial membrane is not permeable to long-chain fatty acids; a multistep process is therefore required for these compounds to be used by mitochondria. In the muscle cytoplasm, long-chain fatty acids are first activated by long-chain acyl-CoA synthetase (ACS) to their CoA thioesters. The CoA thioesters are subsequently linked with carnitine by the enzyme carnitine palmitoyltransferase 1 (CPT 1) located on the inner side of the outer mitochondrial membrane. The acylcarnitine form of the long-chain fatty acid, palmitoylcarnitine, is then transferred across the inner mitochondrial membrane by carnitine:acylcarnitine translocase. Once in the mitochondrial matrix, it is converted back to free acyl-CoA derivative and carnitine by carnitine palmitoyltransferase 2 (CPT 2) on the inner side of the inner mitochondrial membrane. Once carnitine is released, the long-chain acyl-CoA derivative enters the beta-oxidation pathway. With every complete cycle, a two-carbon fragment is cleaved, and an acetyl-CoA molecule is released.
ACS: acyl-CoA synthetase; CPT 1: carnitine palmitoyltransferase 1; CACT: carnitine-acylcarnitine translocase; CPT 2: carnitine palmitoyltransferase 2; VLCAD: very-long-chain acyl-CoA dehydrogenase; TFP: trifunctional protein; LCHAD: long-chain 3-hydroxyacyl-CoA dehydrogenase; MCAD: medium-chain acyl-CoA dehydrogenase; SCAD: short-chain acyl-CoA dehydrogenase; M/SCHAD: medium/short-chain 3-hydroxyacyl-coenzyme A dehydrogenase; FADH2: flavin adenine dinucleotide; ETF: electron transfer flavoprotein; ETFDH: electron transfer flavoprotein dehydrogenase; NADH: nicotinamide adenine dinucleotide.
Graphic 59178 Version 8.0

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