RT Book, Section
A1 Vockley, Jerry
A2 Kline, Mark W.
SR Print(0)
ID 1182928966
T1 Disorders of Fatty Acid Oxidation
T2 Rudolph's Pediatrics, 23e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259588594
LK accesspediatrics.mhmedical.com/content.aspx?aid=1182928966
RD 2024/04/23
AB Mitochondrial  fatty  acid  oxidation  provides  the  main  source  of  energy  for  heart  and  skeletal  muscle  and,  through  generation  of  acetyl-coenzyme  A  (CoA),  for  tricarboxylic  acid  (TCA)  cycle  function  and  ketone  body  production;  it  also  provides  energy  for  other  tissues  when  the  supply  of  glucose  is  limited.  Long-chain  fatty  acids  entering  the  cell  are  esterified  with  carnitine  before  being  transported  across  the  mitochondrial  membrane  through  a  series  of  steps  known  as  the  carnitine  cycle.  The  free  CoA  esters  then  undergo  β-oxidation  in  the  mitochondrial  matrix  (Fig.  145-1).  Disorders  that  interfere  with  any  of  these  steps  limit  energy  production  in  heart  and  skeletal  muscle  at  rest  and  reduce  the  ability  of  other  tissues,  including  the  brain,  to  tolerate  a  low-glucose  milieu  during  times  of  increased  energy  demand.