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

Free Radical Biology & Medicine
P K Held, C O Harding

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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Citations

Oct 8, 2011·Journal of Clinical Biochemistry and Nutrition·Junichi FujiiToshihiro Kurahashi
Nov 20, 2014·Transplant International : Official Journal of the European Society for Organ Transplantation·Yang LiuYiming Li
Sep 25, 2015·World Journal of Gastroenterology : WJG·Jia-Zhong WangYi-Ming Li

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