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Biological function of L-carnitine L-carnitine works together with carnitine palmitoyl transferase (CPT) and carnitine-acylcarnitine translocase (CACT) on the mitochondrial membrane to form an efficient transport system (8)

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Isovaleric acid is an intermediate in the catabolism of the branch-chain amino acid, leucine that accumulates as a result of mutations in the gene encoding isovaleryl-CoA dehydrogenase (IVD)