L-2-oxothiazolidine-4-carboxylate supplementation in murine gamma-GT deficiency.
Free Radic Biol Med
; 34(11): 1482-7, 2003 Jun 01.
Article
in En
| MEDLINE
| ID: mdl-12757858
ABSTRACT
gamma-glutamyl transpeptidase (gamma-GT) deficiency in GGT(enu1) mice is associated with glutathionemia, glutathionuria, growth retardation, infertility, lethargy, cataracts, and shortened life span. Total liver glutathione (GSH) content is significantly reduced in gamma-GT-deficient mice due to chronic excessive GSH loss. Oral supplementation of GGT(enu1) 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 gamma-GT-deficient mice were substantially improved following OTZ supplementation. Tissue GSH deficiency is the proximate cause of the phenotypic abnormalities associated with murine gamma-GT deficiency.
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Database:
MEDLINE
Main subject:
Thiazoles
/
Gamma-Glutamyltransferase
/
Glutathione
/
Growth Disorders
/
Liver
Language:
En
Journal:
Free Radic Biol Med
Year:
2003
Type:
Article
Affiliation country:
United States