Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis

W. A. Gahl, N. Bashan, F. Tietze, I. Bernardini, J. D. Schulman

Research output: Contribution to journalArticlepeer-review

233 Scopus citations

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.

Original languageEnglish
Pages (from-to)1263-1265
Number of pages3
JournalScience
Volume217
Issue number4566
DOIs
StatePublished - 1 Jan 1982
Externally publishedYes

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