Decrease in Free Cystine Content of Cultured Cystinotic Fibroblasts by Ascorbic Acid

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Science  13 Dec 1974:
Vol. 186, Issue 4168, pp. 1040-1042
DOI: 10.1126/science.186.4168.1040


The 100-fold increase in free cystine content characteristic of cultured skin fibroblasts from patients with nephropathic cystinosis was decreased more than 50 percent by addition of L-ascorbic acid to the culture medium at concentrations of 0.29 to 2.9 millimolar. Fresh ascorbic acid must be added to the culture medium daily to produce a progressive decrease of the free cystine content of the cells over a 3-day period. Upon removal of ascorbic acid from the medium, the free cystine content returns to its initial value.