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 6725935
T1 Chapter 150. Disorders of Fatty Acid Oxidation
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=6725935
RD 2024/04/19
AB Mitochondrial  fatty  acid  oxidation  provides  the  main  source  of  energy  for  heart  and  skeletal  muscle  and,  by  generating  acetyl-CoA  for  ketone-body  production,  provides  energy  for  other  tissues  when  the  supply  of  glucose  is  limited.  Fatty  acids  entering  the  cell  are  esterified  with  carnitine  before  being  transported  across  the  mitochondrial  membrane  and  being  beta-oxidized  in  the  mitochondrial  matrix  as  CoA  esters  (Fig.  150-1).  Disorders  that  decrease  β-oxidation  by  blocking  cellular  carnitine  uptake  or  by  impairing  entry  of  fatty  acids  into  mitochondria  or  β-oxidation  limit  energy  production  by  heart  and  skeletal  muscle  at  rest  and  lessen  the  ability  of  other  tissues,  including  the  brain,  to  cope  with  a  low-glucose  milieu.