Decrease in free cystine content of cultured cystinotic fibroblasts by ascorbic acid.
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.
D001205:Ascorbic Acid / Q000494:pharmacology* / Q000627:therapeutic use; D002478:Cells, Cultured; D003553:Cystine / Q000378:metabolism*; D003554:Cystinosis / Q000188:drug therapy / Q000378:metabolism*; D004229:Dithiothreitol / Q000494:pharmacology
D003553:Cystine; D001205:Ascorbic Acid; D004229:Dithiothreitol