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Abbreviations IEM: Inborn error of metabolism mFAO: Mitochondrial fatty acid -oxidation CASTOR: Coenzyme A sequestration, toxicity and redistribution CoASH: Free coenzyme A MCAD: Medium-chain acyl-CoA dehydrogenase MCADD: MCAD deficiency ED: Early diagnosis CPT1: Carnitine palmitoyltransferase 1 CPT2: Carnitine palmitoyltransferase 2 CACT: Carnitine acylcarnitine translocase VLCAD: Very-long-chain acyl-CoA dehydrogenase SCAD: Short-chain acyl-CoA dehydrogenase ACAD: Acyl-CoA dehydrogenase CROT: Crotonase M/SCHAD: Medium-/short-chain hydroxyacyl-CoA dehydrogenase MCKAT: Medium-chain ketoacyl-CoA thiolase MTP: Mitochondrial trifunctional protein ACOTcs: Acyl-CoA thioesterase (CoA-sensitive) ACOTcs: Acyl-CoA thioesterase (CoA-insensitive) ETF: Electron-transferring flavoprotein References Ferreira CR, Rahman S, Keller M, Zschocke J

The KD has been utilized as a therapeutic approach for a considerably longer period than BHB, whether BHB presents additional side effects requires further investigation

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