TY  - CHAP
M1  - Book, Section
TI  - Disorders of Branched Chain Amino and Organic Acid Metabolism
A1  - Vockley, Jerry
A2  - Kline, Mark W.
Y1  - 2018
N1  - 
T2  - Rudolph's Pediatrics, 23e
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.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/20
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1182928364
ER  -