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

  • Gahl W
  • Bashan N
  • Tietze F
 et al. 
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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.

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Authors

  • W. A. Gahl

  • N. Bashan

  • F. Tietze

  • I. Bernardini

  • J. D. Schulman

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