RT Book, Section
A1 Schwahn, Bernd Christian
A2 Rudolph, Colin D.
A2 Rudolph, Abraham M.
A2 Lister, George E.
A2 First, Lewis R.
A2 Gershon, Anne A.
SR Print(0)
ID 6724772
T1 Chapter 138. Disorders of Sulfur-Containing Amino Acid Metabolism
T2 Rudolph's Pediatrics, 22e
YR 2011
FD 2011
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-149723-7
LK accesspediatrics.mhmedical.com/content.aspx?aid=6724772
RD 2024/04/20
AB Sulfur-containing  amino  acids  have  various  roles:  They  mediate  the  transfer  of  methyl  groups  for  virtually  all  transmethylation  reactions;  they  provide  reactive  thiol  groups  that  are  needed  for  detoxification  of  endogenous  and  exogenous  substances;  they  help  maintain  the  intracellular  redox  potential;  and  they  are  a  source  of  sulphate.  Thirteen  disorders  of  sulphur  amino  acid  metabolism  have  been  consistently  described,  11  of  them  potentially  leading  to  disease.