Document Detail

L-cysteine and glutathione metabolism are impaired in premature infants due to cystathionase deficiency.
MedLine Citation:
PMID:  7733030     Owner:  NLM     Status:  MEDLINE    
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 premature infants synthetize glutathione from L-methionine (a process dependent on the cystathionase pathway) at a much lower rate than do erythrocytes from group 2 premature or full-term infants. Thus, the metabolic flow through the transsulfuration pathway may be insufficient to meet the glutathione and cysteine requirements of very premature infants.
J Viña; M Vento; F García-Sala; I R Puertes; E Gascó; J Sastre; M Asensi; F V Pallardó
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of clinical nutrition     Volume:  61     ISSN:  0002-9165     ISO Abbreviation:  Am. J. Clin. Nutr.     Publication Date:  1995 May 
Date Detail:
Created Date:  1995-05-30     Completed Date:  1995-05-30     Revised Date:  2009-06-12    
Medline Journal Info:
Nlm Unique ID:  0376027     Medline TA:  Am J Clin Nutr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1067-9     Citation Subset:  AIM; IM    
Departamento de Fisiología, Universidad de Valencia, Spain.
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MeSH Terms
Amino Acids, Sulfur / blood,  metabolism
Cystathionine / blood
Cystathionine gamma-Lyase / analysis,  blood,  deficiency*
Cysteine / blood,  metabolism*
Gestational Age
Glutathione / blood,  metabolism*
Infant, Newborn
Infant, Premature / metabolism*
Liver / enzymology,  metabolism
Methionine / blood,  metabolism
Rats, Wistar
Reg. No./Substance:
0/Amino Acids, Sulfur; 52-90-4/Cysteine; 56-88-2/Cystathionine; 63-68-3/Methionine; 70-18-8/Glutathione; EC gamma-Lyase
Erratum In:
Am J Clin Nutr. 2009 Jun;89(6):1951

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