Cystinosis: Selective Induction of Vacuolation in Fibroblasts by L-Cysteine-D-Penicillamine Disulfide
- 7 August 1970
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 169 (3945) , 595-597
- https://doi.org/10.1126/science.169.3945.595
Abstract
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 vacuolation by the mixed disulfide in cystinotic but not in normal cells furnishes a histological marker for cystinotic fibroblasts.Keywords
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