Document Detail


L-cysteine and glutathione metabolism are impaired in premature infants due to cystathionase deficiency.
MedLine Citation:
PMID:  7733030     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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    
Affiliation:
Departamento de Fisiología, Universidad de Valencia, Spain.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Amino Acids, Sulfur / blood,  metabolism
Animals
Cystathionine / blood
Cystathionine gamma-Lyase / analysis,  blood,  deficiency*
Cysteine / blood,  metabolism*
Female
Gestational Age
Glutathione / blood,  metabolism*
Humans
Infant, Newborn
Infant, Premature / metabolism*
Liver / enzymology,  metabolism
Methionine / blood,  metabolism
Rats
Rats, Wistar
Chemical
Reg. No./Substance:
0/Amino Acids, Sulfur; 52-90-4/Cysteine; 56-88-2/Cystathionine; 63-68-3/Methionine; 70-18-8/Glutathione; EC 4.4.1.1/Cystathionine gamma-Lyase
Comments/Corrections
Erratum In:
Am J Clin Nutr. 2009 Jun;89(6):1951

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Whole-body protein turnover in response to hyperinsulinemia in humans postabsorptively with [15N]gly...
Next Document:  The effect of raw potato starch on energy expenditure and substrate oxidation.