Cystinosis: electron microscopic evidence of lysosomal storage of cystine in lymph node

A. D. Patrick, B. D. Lake

Department of Chemical Pathology, Institute of Child Health, London
Department of Morbid Anatomy, Institute of Child Health, London

Electron microscopic examination of a biopsy specimen of cystinotic lymph node demonstrated that cystine crystals were entirely intracellular, and that the majority, including those of the largest size, were enclosed by intact limiting membranes. Acid phosphatase activity was localized specifically at the periphery of the crystal profiles and the smallest crystal aggregates were contained in osmophilic dense bodies which had the appearance of progressively swollen lysosomes. The implications of the suggested lysosomal distribution of cystine are discussed.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Haq, M. R., Kalatzis, V., Gubler, M.-C., Town, M. M., Antignac, C., van't Hoff, W. G., Woolf, A. S. (2002). Immunolocalization of Cystinosin, the Protein Defective in Cystinosis. J. Am. Soc. Nephrol. 13: 2046-2051 [Abstract] [Full Text]  
• Kleta, R., Gahl, W. A. (2002). Cystinosis: Antibodies and Healthy Bodies. J. Am. Soc. Nephrol. 13: 2189-2191 [Full Text]  
• Gahl, W. A., Thoene, J. G., Schneider, J. A., O'Regan, S., Kaiser-Kupfer, M. I., Kuwabara, T. (1988). Cystinosis: Progress in a Prototypic Disease. ANN INTERN MED 109: 557-569 [Abstract]  
• SCHULMAN, J. D., MUDD, S. H., SCHNEIDER, J. A., SPIELBERG, S. P., BOXER, L., OLIVER, J., CORASH, L., SHEETZ, M. (1980). Genetic Disorders of Glutathione and Sulfur Amino-Acid Metabolism: New Biochemical Insights and Therapeutic Approaches. ANN INTERN MED 93: 330-346 [Abstract]  
• Kroll, W. A., Schneider, J. A. (1974). Decrease in Free Cystine Content of Cultured Cystinotic Fibroblasts by Ascorbic Acid. Science 186: 1040-1042 [Abstract]  
• Sanderson, P. O., Kuwabara, T., Stark, W. J., Wong, V. G., Collins, E. M. (1974). Cystinosis: A Clinical, Histopathologic, and Ultrastructural Study. Arch Ophthalmol 91: 270-274 [Abstract]  
• Schulman, J. D., Bradley, K. H. (1970). Cystinosis: Selective Induction of Vacuolation in Fibroblasts by L-Cysteine-D-Penicillamine Disulfide. Science 169: 595-597 [Abstract]  
• Hummeler, K., Zajac, B. A., Genel, M., Holtzapple, P. G., Segal, S. (1970). Human Cystinosis: Intracellular Deposition of Cystine. Science 168: 859-860 [Abstract]  
• Schulman, J. D., Wong, V. G., Kuwabara, T., Bradley, K. H., Seegmiller, J. E. (1970). Intracellular Cystine Content of Leukocyte Populations in Cystinosis. Arch Intern Med 125: 660-664 [Abstract]  
• Schulman, J. D., Bradley, K. H., Seegmiller, J. E. (1969). Cystine: Compartmentalization within Lysosomes in Cystinotic Leukocytes. Science 166: 1152-1154 [Abstract]