[HTML][HTML] Impact of peripheral ketolytic deficiency on hepatic ketogenesis and gluconeogenesis during the transition to birth

DG Cotter, B Ercal, DA d'Avignon, DJ Dietzen… - Journal of Biological …, 2013 - ASBMB
Preservation of bioenergetic homeostasis during the transition from the carbohydrate-laden
fetal diet to the high fat, low carbohydrate neonatal diet requires inductions of hepatic fatty
acid oxidation, gluconeogenesis, and ketogenesis. Mice with loss-of-function mutation in the
extrahepatic mitochondrial enzyme CoA transferase (succinyl-CoA: 3-oxoacid CoA
transferase, SCOT, encoded by nuclear Oxct1) cannot terminally oxidize ketone bodies and
develop lethal hyperketonemic hypoglycemia within 48 h of birth. Here we use this model to …