RT Book, Section
A1 Goodman, Stephen I.
A2 Rudolph, Colin D.
A2 Rudolph, Abraham M.
A2 Lister, George E.
A2 First, Lewis R.
A2 Gershon, Anne A.
SR Print(0)
ID 6726015
T1 Chapter 151. Disorders of Ketogenesis
T2 Rudolph's Pediatrics, 22e
YR 2011
FD 2011
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-149723-7
LK accesspediatrics.mhmedical.com/content.aspx?aid=6726015
RD 2024/04/19
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.