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

American Journal of Clinical Nutrition

Volumn 61, Issue 5, 1995, Pages 1067-1069

  Viña, José ^a   Vento, Máxima ^b   García Sala, Fernando ^b   Puertes, Inmaculada R ^b   Gascó, Esperanza ^b   Sastre, Juan ^b   Asensi, Miguel ^b   Pallardó, Federico V ^b  

^a UNIVERSITY OF VALENCIA   (Spain)

^b NONE

Author keywords

amino acids; glutathione; humans; N acetyl cysteine; parenteral nutrition; Transsulfuration

Indexed keywords

CYSTATHIONINE GAMMA LYASE; CYSTEINE; GLUTATHIONE; SULFUR AMINO ACID;

AMINO ACID METABOLISM; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME BLOOD LEVEL; ENZYME DEFICIENCY; GLUTATHIONE METABOLISM; HUMAN; INBORN ERROR OF METABOLISM; INFANT; PARENTERAL NUTRITION; PREMATURITY;

EID: 0029007480     PISSN: 00029165     EISSN: None     Source Type: Journal    
DOI: 10.1093/ajcn/61.5.1067     Document Type: Article

References (22)

1. Sturman JA, Gaull G, Raiha NCR. Absence of cystathionase in human fetal liver: is cystine essential? Science 1970;169:74-5.
2. Zlotkin SH, Anderson H. The development of cystathionase activity during the first year of life. Pediatr Res 1982;16:65-8.
3. Rigo J, Senterre J. Parenteral nutrition in the very-low-birth-weight infant. In: Kretchmer N, Minkowski A, eds. Nutritional adaptation of gastrointestinal tract of the newborn. New York: Raven Press, 1983: 191-207.
4. Rao AM, Draake MR, Stipanuk MH. Role of the transsulfuration pathway and of cystathionase activity in the formation of cysteine and sulphate from methionine in rat hepatocytes. J Nutr 1990;120:837-45.
5. Cho ES, Hovanecbrown J, Tomanek RJ, Stegink LD. Propargylglycine infusion effects on tissue glutathione levels, plasma amino acid concentrations and tissue morphology in parenterally-fed growing rats. J Nutr 1991;121:785-94.
6. Jones MR, Cheek JM, Tamaki J, Edmond J, Wu PYK. Plasma amino acid concentration in premature infants: effect of sampling site. Am J Clin Nutr 1989;50:1389-94.
7. Obreholzer VG, Briddon A. A novel use of amino acid ratios as an indicator of nutritional status. In: Lubec G, Rosenthal GA, eds. Amino acids: chemistry, biology and medicine. Leiden, Netherlands: Escom Science Publishers, 1990:1079-83.
8. Viña J, Hems R, Krebs HA. Maintenance of glutathione content in isolated hepatocytes. Biochem J 1978;170:627-30.
9. Beatty P, Reed DJ. Involvement of the cystathionine pathway in the biosynthesis of glutathione by isolated rat hepatocytes. Arch Biochem Biophys 1980;204:80-7.
10. Dubowitz L, Dubowitz V, Goldberg C. Clinical assessment of gestational age in the newborn infant. J Pediatr 1970;77:1-8.
11. Viña J, Puertes IR, Estrela JM, Viña JR, Galbis JL. Involvement of gamma glutamyl transferase in amino acid uptake by the lactating mammary gland of the rat. Biochem J 1981;194:99-102.
12. Krebs H, Henseleit K. Research on urea synthesis in animal bodies. (Untersuchungen über die Harnstoffbildung im Tierkörper Hoppe-Seyler's.) Z Physiol Chem 1932;210:33-66 (in German).
13. Boyland E, Chasseaud LF. Enzyme-catalysed conjugations of glutathione with unsaturated compounds. Biochem J 1967;104:95-102.
14. Viña JR, Puertes IR, Rodriguez A, Sáez GT, Viña J. Effect of physiological starvation on amino acid metabolism by lactating mammary gland. Studies in women and rats. J Nutr 1987;117:533-8.
15. National Research Council. Guide for the care and use of laboratory animals. Bethesda, MD: National Institutes of Health, 1985.
16. Heinonen K. Studies on cystathionase activity in rat liver and brain during development. Effects of hormones and amino acids in vivo. Biochem J 1973;136:1011-5.
17. Viña J, Gimenez A, Puertes IR, Gascó E, Viña JR. Impairement of cysteine synthesis from methionine in rats exposed to surgical stress. Br J Nutr 1992;68:421-9.
18. Bancalari E, Stocker JT, eds. Bronchopulmonary dysplasia. Cambridge, UK: Hemisphere, 1988.
19. Sies H. Biochemistry of oxidative stress. Angew Chem 1986;25:1058-71.
20. Shahal Y, Bauminger ER, Zmora E, et al. Oxidative stress in newborn erythrocytes. Pediatr Res 1991;29:119-22.
21. Phelps DL. Neonatal oxygen toxicity. Is it preventable? Pediatr Clin North Am 1982;29:1233-41.
22. Frank L. Antioxidants, nutrition and bronchopulmonary displasia. In: Holtzman RB, Frank L, eds. Clin Perinatol 1992;19:541-62.