Cystine Transport Is Defective in Isolated Leukocyte Lysosomes from Patients with Cystinosis
- 24 September 1982
- journal article
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 217 (4566), 1263-1265
- https://doi.org/10.1126/science.7112129
Abstract
The activity of a cystine transport system in lysosomes prepared from the leukocytes of patients with cystinosis was found to be deficient. In normal subjects, this system was resistant to N-ethylmaleimide and demonstrated saturation kinetics. Lysosomes from individuals heterozygous for cystinosis demonstrated a reduced maximum velocity for cystine egress from lysosomes. The rate of cystine escape from normal lysosomes was enhanced by adenosine triphosphate. The availability of normal and mutant lysosomes provides a means of investigating mechanisms of amino acid transport across lysosomal membranes.Keywords
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