Human Cystinosis: Intracellular Deposition of Cystine
- 15 May 1970
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 168 (3933), 859-860
- https://doi.org/10.1126/science.168.3933.859
Abstract
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.Keywords
This publication has 5 references indexed in Scilit:
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- Diagnosis of Cystinosis by Rectal BiopsyNew England Journal of Medicine, 1969
- Cystinosis: electron microscopic evidence of lysosomal storage of cystine in lymph nodeJournal of Clinical Pathology, 1968
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- Increased Cystine in Leukocytes from Individuals Homozygous and Heterozygous for CystinosisScience, 1967