S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency

Journal of Inherited Metabolic Disease

Volumn 27, Issue 6, 2004, Pages 783-786

  Okun, J G ^a   Sauer, S ^a   Bahr S ^a   Lenhartz, H ^a   Mayatepek, E ^b  

^a UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^b UNIVERSITY HOSPITAL DÜSSELDORF   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLGLUTATHIONE; GLUTATHIONE DERIVATIVE; GLUTATHIONE SYNTHASE; UNCLASSIFIED DRUG;

ARTICLE; AUTOSOMAL RECESSIVE INHERITANCE; CONTROLLED STUDY; CULTURE MEDIUM; DRUG MECHANISM; ENZYME ACTIVATION; ENZYME SUBSTRATE; FIBROBLAST CULTURE; GLUTATHIONE SYNTHASE DEFICIENCY; HUMAN; HUMAN CELL; INBORN ERROR OF METABOLISM; INTRACELLULAR SPACE; PATHOGENESIS; TREATMENT PLANNING;

CELLS, CULTURED; FIBROBLASTS; GLUTATHIONE; GLUTATHIONE SYNTHASE; HUMANS; METABOLISM, INBORN ERRORS; SKIN;

EID: 7244238102     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/B:BOLI.0000045838.65498.ff     Document Type: Article

References (9)

1. Anderson ME (1985) Determination of glutathione and glutathione disulfide in biological samples. Methods Enzymol. 113: 548-555.
2. Anderson ME, Meister A (1980) Dynamic state of glutathione in blood plasma. J Biol Chem 255: 9530-9533.
3. Boxer LA, Oliver JM, Spielberg SP, Allen JM, Schulman JD (1979) Protection of granulocytes by vitamin E in glutathione synthetase deficiency. N Engl J Med 301: 901-905.
4. Galzigna L, Rizzoli V, Schiapellu P, Moretto C (1994) S-Acetyl and S-phenylacetyl-glutathione as glutathione precursors in rat plasma and tissue preparations. Enzyme Protein 48: 98-104.
5. Jain A, Buist NR, Kennaway NG, Powell BR, Auld PA, Martensson J (1994) Effect of ascorbate or N-acetyleysteine treatment in a patient with hereditary glutathione synthetase deficiency. J Pediatr 124: 229-233.
6. Meister A, Anderson ME (1983) Glutathione. Annu Rev Biochem 52 711-760.
7. Ristoff E, Mayatepek E, Larsson A (2001) Long-term clinical outcome in patients with glutathione synthetase deficiency. J Pediatr 139 79-84.
8. Ristoff E, Herbert C, Njalsson R, Norgren S, Rooyackers O, Larsson A (2002) Glutathione synthetase deficiency: Is gamma glutamylcysteine accumulation a way to cope with oxidative stress in cells with insufficient levels of glutathione? J Inherit Metab Dis 25: 577-584.
9. Vogel JU, Cinatl J, Dauletbaev N, et al (2003) Effects of S acetylglutathione in cell and animal model of herpes simplex virus type 1 infection. Med Microbiol Immunol [Epub ahead of print]