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 language | English (US) |
---|---|
Pages (from-to) | 1482-1487 |
Number of pages | 6 |
Journal | Free Radical Biology and Medicine |
Volume | 34 |
Issue number | 11 |
DOIs | |
State | Published - 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)