Search results for "Cystathioninuria"

L-cysteine and glutathione metabolism are impaired in premature infants due to cystathionase deficiency.

1995

There are conflicting reports in the literature as to whether L-cysteine is an essential amino acid in premature infants as the result of the absence of hepatic cystathionase activity. To analyze the physiological importance of the cystathionase deficiency, we studied sulfur amino acid metabolism in human neonates of different gestational ages. Plasma cystathionine concentrations are higher in premature infants < or = 32 wk gestation (group 1) than in premature infants of 33-36 wk gestational age (group 2) or in full-term infants (group 3), whereas plasma cysteine concentrations are much lower in group 1 and 2 premature infants than in mature infants. Furthermore, erythrocytes from group 1 …