RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164060644
T1 Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHADD)
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164060644
RD 2024/04/17
AB The  mitochondrial  trifunctional  protein  (MTP)  is  located  on  the  inner  mitochondrial  membrane  and  catalyzes  three  of  the  four  chain-shortening  reactions  in  the  mitochondrial  β-oxidation  of  long-chain  fatty  acids.  The  three  enzymes  involved  in  these  reactions  are  2-enoyl  coenzyme  A  (CoA)  hydratase,  long-chain  3-ketoacyl  CoA  thiolase,  and  long-chain  3-hydroxy  acyl-coenzyme  A  dehydrogenase  (LCHAD),  which  catalyzes  the  conversion  of  long-chain  3-hydroxyacyl-CoA  esters  into  3-ketoacyl-CoA  esters.