Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide.

@article{Schulman1970CystinosisSI,
  title={Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide.},
  author={Joseph D. Schulman and Kathryn H. Bradley},
  journal={Science},
  year={1970},
  volume={169 3945},
  pages={595-7}
}
In cultured fibroblasts from individuals with cystinosis vacuolation is induced by exposure to L-cysteine-D-penicillamine disulfide. Normal fibroblasts do not show vacuolation on such exposure. These observations provide direct evidence that cystinotic cells have deficient activity of a lysosomal system for disulfide metabolism or transport. Induction of… CONTINUE READING