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

  • Published:
Journal of Inherited Metabolic Disease

Abstract

Summary: Glutathione synthetase deficiency is an autosomal recessive inherited metabolic defect in the γ-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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Authors and Affiliations

  1. Department of General Pediatrics, University Children's Hospital, Heidelberg, Germany

    J. G. Okun, S. Sauer, S. Bähr & H. Lenhartz

  2. Department of General Pediatrics, University Children's Hospital, Düsseldorf, Germany

    E. Mayatepek

Authors
  1. J. G. Okun
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  2. S. Sauer
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  3. S. Bähr
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  4. H. Lenhartz
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  5. E. Mayatepek
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Corresponding author

Correspondence to E. Mayatepek.

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Okun, J.G., Sauer, S., Bähr, S. et al. S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency. J Inherit Metab Dis 27, 783–786 (2004). https://doi.org/10.1023/B:BOLI.0000045838.65498.ff

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  • DOI: https://doi.org/10.1023/B:BOLI.0000045838.65498.ff

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