title: Effects of decreased availability of sulfur amino acids in severe childhood undernutrition.
abstract: In studies of glutathione (GSH) metabolism in children with severe childhood undernutrition (SCU), slower erythrocyte GSH synthesis in children with edema was associated with lower concentrations of cysteine, the rate-limiting precursor of GSH synthesis. This finding suggested a shortage of cysteine available for GSH synthesis in children with edematous SCU. The plasma concentration of methionine, the sulfur donor for cysteine synthesis, was also lower in children with edematous SCU, suggesting decreased availability of methionine for cysteine synthesis. It is also possible that reduced methionine availability will result in decreased synthesis of S-adenosylmethionine, which could lead to an overall defect in methylation reactions. This review focuses on the relationship between cysteine availability and GSH synthesis in children with SCU. It also examines whether there is an inadequate supply of cysteine in those with edematous SCU and, if so, whether this is due to a shortage of methionine due to a decreased release of methionine from protein breakdown. Finally, the review explores whether a shortage of methionine results in decreased synthesis of S-adenosylmethionine, the universal methyl donor.
authors.affiliation: USDA/Agricultural Research Service, Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, 1100 Bates Street, Houston, TX 77030-2600, USA. fjahoor@bcm.tmc.edu
authors.forename: Farook
authors.initials: F
authors.lastname: Jahoor
mesh_terms: D000603:Amino Acids, Sulfur / Q000493:pharmacokinetics*; D001682:Biological Availability; D002648:Child; D015362:Child Nutrition Disorders / Q000150:complications / Q000378:metabolism*; D002675:Child, Preschool; D004487:Edema / Q000150:complications / Q000378:metabolism; D005978:Glutathione / Q000378:metabolism*; D006710:Homocysteine / Q000378:metabolism; D006801:Humans; D008715:Methionine / Q000096:biosynthesis / Q000172:deficiency*; D008745:Methylation; D011502:Protein-Energy Malnutrition / Q000150:complications / Q000378:metabolism
chemical_list: D000603:Amino Acids, Sulfur; D006710:Homocysteine; D008715:Methionine; D005978:Glutathione