TY  - CHAP
M1  - Book, Section
TI  - Catabolism of the Carbon Skeletons of Amino Acids
A1  - Rodwell, Victor W.
A2  - Rodwell, Victor W.
A2  - Bender, David A.
A2  - Botham, Kathleen M.
A2  - Kennelly, Peter J.
A2  - Weil, P. Anthony
PY  - 2018
T2  - Harper's Illustrated Biochemistry, 31e
AB  - OBJECTIVESAfter  studying  this  chapter,  you  should  be  able  to:Name  the  principal  catabolites  of  the  carbon  skeletons  of  the  protein  amino  acids  and  the  major  metabolic  fates  of  these  catabolites.Write  an  equation  for  an  aminotransferase  (transaminase)  reaction  and  illustrate  the  role  played  by  the  coenzyme.Outline  the  metabolic  pathways  for  each  of  the  protein  amino  acids,  and  identify  reactions  associated  with  clinically  significant  metabolic  disorders.Provide  examples  of  aminoacidurias  that  arise  from  defects  in  glomerular  tubular  reabsorption,  and  the  consequences  of  impaired  intestinal  absorption  of  tryptophan.Explain  why  metabolic  defects  in  different  enzymes  of  the  catabolism  of  a  specific  amino  acid  can  be  associated  with  similar  clinical  signs  and  symptoms.Describe  the  implications  of  a  metabolic  defect  in  Δ1-pyrroline-5-carboxylate  dehydrogenase  for  the  catabolism  of  proline  and  of  4-hydroxyproline.Explain  how  the  α-amino  nitrogen  of  proline  and  of  lysine  is  removed  by  processes  other  than  transamination.Draw  analogies  between  the  reactions  that  participate  in  the  catabolism  of  fatty  acids  and  of  the  branched-chain  amino  acids.Identify  the  specific  metabolic  defects  in  hypervalinemia,  maple  syrup  urine  disease,  intermittent  branched-chain  ketonuria,  isovaleric  acidemia,  and  methylmalonic  aciduria.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/20
UR  - accesspharmacy.mhmedical.com/content.aspx?aid=1162228887
ER  -