MICE WITH GENETIC GAMMA-GLUTAMYL-TRANSPEPTIDASE DEFICIENCY EXHIBIT GLUTATHIONURIA, SEVERE GROWTH FAILURE, REDUCED LIFE SPANS, AND INFERTILITY

A mouse mutant with glutathionuria was discovered by screening for ami no acidurias in the progeny of ethylnitrosourea-mutagenized mice. Tota l 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 i nfertility, gamma-Glutamyl transpeptidase activity was deficient in ki dney 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 gamma-glutamyl transpeptidase physiology and glutathione metabolism.