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

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.

• Authors: J D Schulman, K H Bradley
• Journal: Science (New York, N.Y.) (Science) Vol. 169 Issue 3945 Pg. 595-7 (Aug 07 1970) ISSN: 0036-8075 [Print] United States
• PMID: 4913301 (Publication Type: Journal Article)

Chemical References

• Sulfides
• Cystine
• Penicillamine
• Cysteine

Topics

• Biological Transport
• Culture Techniques
• Cysteine (pharmacology)
• Cystine (metabolism)
• Cystinosis (pathology)
• Cytoplasm
• Fibroblasts (cytology, drug effects)
• Humans
• Lysosomes (metabolism)
• Microscopy, Phase-Contrast
• Penicillamine
• Sulfides (metabolism, pharmacology)