Mice with genetic γ-glutamyl transpeptidase deficiency exhibit glutathionuria, severe growth failure, reduced life spans, and infertility

Cary Harding, Phillip Williams, Elizabeth Wagner, Dominica S. Chang, Krzysztof Wild, Robert E. Colwell, Jon A. Wolff

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A mouse mutant with glutathionuria was discovered by screening for amino acidurias in the progeny of ethylnitrosourea-mutagenized mice. Total glutathione concentration was increased in both blood and urine but decreased in liver homogenates from affected mice. Glutathionuric mice exhibited lethargy, severe growth failure, shortened life spans and infertility. γ- Glutamyl transpeptidase activity was deficient in kidney homogenates of glutathionuric mice. The glutathionuric phenotype in these mice is inherited as an autosomal recessive trait. This mouse mutant will be a useful animal model for the study of γ-glutamyl transpeptidase physiology and glutathione metabolism.

Original languageEnglish (US)
Pages (from-to)12560-12567
Number of pages8
JournalJournal of Biological Chemistry
Issue number19
Publication statusPublished - May 9 1997
Externally publishedYes


ASJC Scopus subject areas

  • Biochemistry

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