TY  - CHAP
M1  - Book, Section
TI  - Chapter 137. Disorders of Branched Chain Amino and Organic Acid Metabolism
A1  - Goodman, Stephen I.
A1  - Saudubray, Jean-Marie
A2  - Rudolph, Colin D.
A2  - Rudolph, Abraham M.
A2  - Lister, George E.
A2  - First, Lewis R.
A2  - Gershon, Anne A.
PY  - 2011
T2  - Rudolph's Pediatrics, 22e
AB  - The  three  essential  branched  chain  aminoacids  (BCAAs),  leucine,  isoleucine,  and  valine  encompass  about  25%  of  human  protein.  They  are  metabolized  in  mitochondria.  The  first  two  catabolic  steps  are  common  to  the  three  BCAAs  (Fig.  137-1).  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,  is  also  highly  regulated  by  a  kinase/phosphatase  system  and  plays  a  key  role  in  nitrogen  metabolism.  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=6724685
ER  -