Abstract
Cultured skin fibroblasts from patients with 5-oxoprolinuria caused by hereditary deficiency of glutathione synthetase have decreased levels of the corresponding enzyme as well as of glutathione. Fibroblasts from the same patients accumulated γ-glutamyl cysteine, but the levels were lower than those of glutathione in control fibroblasts. The uptake of [ 35S]cystine was equally rapid in control and patient fibroblasts. In the acid-soluble fraction γ-glutamyl-[ 35S]cysteine accumulated in fibroblasts from patients but not from controls. Appreciable turnover of γ-glutamyl cysteine and glutathione in the respective cell strains was observed, the half-lives of these pools being approximately 5 hours. The growth rate of mutant fibroblasts in culture was significantly slower than that of control fibroblasts. There was no significant accumulation of 5-oxoproline in the culture medium.
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