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CPT-1B is the muscle isoform and is highly expressed in heart, skeletal muscle and testis [2]

Malonyl coenzyme A decarboxylase deficiency

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The transport system consists of three main proteins, the first of which is the enzyme carnitine palmitoyltransferase I (CPTI) present in the mitochondrial outer membrane that complexes with a long-chain acyl-CoA synthetase and the voltage-dependent anion channel, while the second transport component is carnitine:acylcarnitine translocase (CACT), an integral inner membrane protein, which complexes with carnitine palmitoyltransferase II (CPTII) located on the matrix side of the inner membrane