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

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

doi:10.1126/science.7112129

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

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

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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.

Original language	English
Pages (from-to)	1263-1265
Number of pages	3
Journal	Science
Volume	217
Issue number	4566
DOIs	https://doi.org/10.1126/science.7112129
State	Published - 1 Jan 1982
Externally published	Yes

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title = "Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis",

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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AU - Gahl, W. A.

AU - Bashan, N.

AU - Tietze, F.

AU - Bernardini, I.

AU - Schulman, J. D.

PY - 1982/1/1

Y1 - 1982/1/1

N2 - 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.

AB - 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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U2 - 10.1126/science.7112129

DO - 10.1126/science.7112129

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AN - SCOPUS:0019940282

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SP - 1263

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JO - Science

JF - Science

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ER -

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

Abstract

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