Transcriptome analysis suggests a compensatory role of the cofactors coenzyme A and NAD⁺ in medium-chain acyl-CoA dehydrogenase knockout mice.

ABSTRACT: During fasting, mitochondrial fatty-acid β-oxidation (mFAO) is essential for the generation of glucose by the liver. Children with a loss-of-function deficiency in the mFAO enzyme medium-chain acyl-Coenzyme A dehydrogenase (MCAD) are at serious risk of life-threatening low blood glucose levels during fasting in combination with intercurrent disease. However, a subset of these children remains asymptomatic throughout life. In MCAD-deficient (MCAD-KO) mice, glucose levels are similar to those of wild-type (WT) mice, even during fasting. We investigated if metabolic adaptations in the liver may underlie the robustness of this KO mouse. WT and KO mice were given a high- or low-fat diet and subsequently fasted. We analyzed histology, mitochondrial function, targeted mitochondrial proteomics, an

SUBMITTER: Martines AMF 

PROVIDER: S-EPMC6787083 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature