RT Book, Section
A1 Vockley, Jerry
A2 Kline, Mark W.
SR Print(0)
ID 1182928364
T1 Disorders of Branched Chain Amino and Organic Acid Metabolism
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=1182928364
RD 2024/04/25
AB The  3  essential  branched  chain  amino  acids  (BCAAs),  leucine,  isoleucine,  and  valine,  encompass  about  25%  of  human  protein.  They  are  metabolized  in  mitochondria.  The  first  2  catabolic  steps  are  common  to  the  3  BCAAs  (Fig.  132-1).  The  first  reaction,  which  occurs  primarily  in  muscle,  involves  reversible  transamination  to  2-oxo  (or  keto)  acids  and  is  followed  by  oxidative  decarboxylation  to  coenzyme  A  (CoA)  derivatives  by  branched  chain  oxo  (or  keto)  acid  dehydrogenase  (BCKD).  BCKD  is  similar  in  structure  to  pyruvate  dehydrogenase  (and  shares  a  common  subunit),  is  also  highly  regulated  by  a  kinase/phosphatase  system,  and  plays  a  key  role  in  nitrogen  metabolism.