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Moreover, mitochondria in patient NPs displayed larger individual volume, lower sphericity, and an increased number of network junctions (Fig

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This regulation during fasting entails adenosine monophosphate-activated protein kinase (AMPK)-mediated inhibition of acetyl-coenzyme A (acetyl-CoA) carboxylase alpha (ACACA), the rate-limiting enzyme of fatty acid synthesis, while during feeding, fatty acid synthesis metabolite and ACACA reaction product malonyl-CoA inhibits mitochondrial carnitine palmitoyltransferase 1A (CPT1A), the rate-limiting enzyme of fatty acid beta-oxidation (Figure 1)