Fig.3. Loss of ketone body production impairs mitochondrial function.

When ketogenesis is impaired, the substrate acetyl-CoA accumulates. This accumulated acetyl-CoA then leads to ectopic fat deposition through de novo lipogenesis, and within the mitochondria, excessive protein acetylation progresses, causing functional impairment. AcAc, acetoacetate; AcAc-CoA, acetoacetyl-CoA; βOHB, β-hydroxybutyrate; NAD, nicotinamide adenine dinucleotide