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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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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

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Under stress conditions, mitochondrial damage results in the production of excessive mtROS, provoking oxidative damage to mtDNA and mtDNA release into the cytoplasm through the mitochondrial permeability transition pore, which further activates the DNA sensor cGAS-STING and facilitates downstream inflammatory processes (101, 102)

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