RT Book, Section
A1 Yudkoff, Marc
A1 Summar, Marshall L.
A1 Häberle, Johannes
A2 Sarafoglou, Kyriakie
A2 Hoffmann, Georg F.
A2 Roth, Karl S.
SR Print(0)
ID 1140316009
T1 Urea Cycle Disorders
T2 Pediatric Endocrinology and Inborn Errors of Metabolism, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071773140
LK accesspediatrics.mhmedical.com/content.aspx?aid=1140316009
RD 2024/04/19
AB Essentially  all  animal  cells  form  ammonia  in  the  course  of  the  metabolism  of  amino  acids  and  the  adenine  nucleotides.  Mammals  prevent  the  accumulation  of  ammonia,  which  is  neurotoxic  in  high  concentration,  by  converting  this  compound  to  urea  via  the  urea  cycle  (Figure  13-1),  which  was  “discovered”  in  1932  by  Krebs  and  Henseleit.1  Ureagenesis  is  an  extremely  efficient  mechanism  for  the  disposal  of  waste  nitrogen.  A  healthy  adult  who  daily  consumes  50  to  100  grams  of  protein  will  excrete  10  to  20  grams  of  nitrogen  (N)  as  urea,  or  80%  to  90%  of  total  urinary  N.