TY - CHAP M1 - Book, Section TI - Disorders of Ketogenesis A1 - Vockley, Jerry A2 - Kline, Mark W. PY - 2018 T2 - Rudolph's Pediatrics, 23e AB - Hepatic biosynthesis of ketone bodies involves the condensation of acetyl-coenzyme A (CoA) and acetoacetyl-CoA to hydroxymethylglutaryl-CoA (HMG-CoA) by HMG-CoA synthase, followed by hydrolysis of HMG-CoA to acetyl-CoA and acetoacetic acid (AcAc) by HMG-CoA lyase as the final step in leucine degradation. AcAc is reduced to 3-hydroxybutyric acid (3HB), and extrahepatic tissues use the 2 ketone bodies as energy sources during fasting (ketolysis). Recessively inherited defects of HMG-CoA synthase (HMGCS2) and HMG-CoA lyase (HMGCL) cause hypoketotic hypoglycemia during fasting, and defects of ketolysis cause persistent or episodic ketoacidosis. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1182928979 ER -