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Science 15 May 1970:
Vol. 168. no. 3933, pp. 859 - 860
DOI: 10.1126/science.168.3933.859
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Articles

Human Cystinosis: Intracellular Deposition of Cystine

Klaus Hummeler 1, Barbara A. Zajac 1, Myron Genel 1, Philip G. Holtzapple 1, and Stanton Segal 1

1 Department of Pediatrics, Medical School, University of Pennsylvania, and Children's Hospital of Philadelphia, Philadelphia 19146

Membrane-limited inclusions were found in the cytoplasm of cells of the rectal mucosa, leukocytes, and cultured fibroblasts from two humans with cystinosis. Most of the inclusions contained amorphous material, presumably cystine. In cells of the rectal mucosa the material appeared frequently crystallized. This was rarely seen in leukocytes, and never in cultured fibroblasts. The fact that acid phosphatase could be demonstrated consistently in the organelles responsible for sequestration of cystine indicates that they are lysosomes.


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Cystinosis: Selective Induction of Vacuolation in Fibroblasts by L-Cysteine-D-Penicillamine Disulfide.
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Science. ISSN 0036-8075 (print), 1095-9203 (online)