L-2-oxothiazolidine-4-carboxylate supplementation in murine γ-GT deficiency

Patrice Held, Cary O. Harding

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

Abstract

γ-glutamyl transpeptidase (γ-GT) deficiency in GGTenu1 mice is associated with glutathionemia, glutathionuria, growth retardation, infertility, lethargy, cataracts, and shortened life span. Total liver glutathione (GSH) content is significantly reduced in γ-GT-deficient mice due to chronic excessive GSH loss. Oral supplementation of GGTenu1 mice with L-2-oxothiazolidine-4-carboxylate (OTZ), a cysteine prodrug, led to partial restoration of liver GSH content. The growth, physical appearance, and behavior of γ-GT-deficient mice were substantially improved following OTZ supplementation. Tissue GSH deficiency is the proximate cause of the phenotypic abnormalities associated with murine γ-GT deficiency.

Original languageEnglish (US)
Pages (from-to)1482-1487
Number of pages6
JournalFree Radical Biology and Medicine
Volume34
Issue number11
DOIs
StatePublished - Jun 1 2003

Keywords

  • Cysteine
  • Free radicals
  • Glutathione
  • L-2-oxothiazolidine-4-carboxylate
  • Mouse model
  • γ-Glutamyl transpeptidase

ASJC Scopus subject areas

  • Biochemistry
  • Physiology (medical)

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