TY - CHAP M1 - Book, Section TI - Chapter 151. Disorders of Ketogenesis A1 - Goodman, Stephen I. A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. PY - 2011 T2 - Rudolph's Pediatrics, 22e AB - Hepatic biosynthesis of ketone bodies involves the condensation of acetyl-CoA and acetoacetyl-CoA to hydroxymethylglutaryl-CoA (HMG-CoA) by HMG-CoA synthetase, followed by hydrolysis of HMG-CoA to acetyl-CoA and acetoacetic acid (AcAc) by HMG-CoA lyase. AcAc is reduced to 3-hydroxybutyric acid (3HB), and extrahepatic tissues use the two ketone bodies as energy sources during fasting (ketolysis). Recessively inherited defects of HMG-CoA synthetase and HMG-CoA lyase cause hypoketotic hypoglycemia during fasting, and defects of ketolysis cause episodic ketoacidosis. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=6726015 ER -