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Science 7 August 1970:
Vol. 169. no. 3945, pp. 595 - 597
DOI: 10.1126/science.169.3945.595
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Articles

Cystinosis: Selective Induction of Vacuolation in Fibroblasts by L-Cysteine-D-Penicillamine Disulfide

Joseph D. Schulman 1 and Kathryn H. Bradley 1

1 National Institute of Arthritis and Metabolic Diseases, Bethesda, Maryland

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
'Adolescent' Cystinosis: A Case Presentation and Review of the Recent Literature.
T. J. Zimmerman, C. I. Hood, and A. R. Gasset (1974)
Arch Ophthalmol 92, 265-268
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