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

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

  • J Inherit Metab Dis. 2004;27(6):783-6. doi: 10.1023/b:boli.0000045838.65498.ff.
J G Okun 1,  S Sauer,  S Bähr,  H Lenhartz,  E Mayatepek
Affiliations

Affiliation

  • 1 Department of General Pediatrics, University Children's Hospital, Heidelberg, Germany.
PMID: 15617191 DOI: 10.1023/b:boli.0000045838.65498.ff
Abstract

Glutathione synthetase deficiency is an autosomal recessive inherited metabolic defect in the gamma-glutamyl cycle. Decreased intracellular glutathione levels are one of the characteristic biochemical features. In this study we show that addition of S-acetylglutathione to the medium raised intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency. This has implications for the treatment of patients with this inborn error of metabolism.

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