| Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis. | |
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MedLine Citation:
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PMID: 7112129 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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:
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W A Gahl; N Bashan; F Tietze; I Bernardini; J D Schulman |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Science (New York, N.Y.) Volume: 217 ISSN: 0036-8075 ISO Abbreviation: Science Publication Date: 1982 Sep |
Date Detail:
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Created Date: 1982-10-21 Completed Date: 1982-10-21 Revised Date: 2007-03-19 |
Medline Journal Info:
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Nlm Unique ID: 0404511 Medline TA: Science Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1263-5 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Biological Transport
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drug effects Carrier Proteins / metabolism Cysteine / metabolism Cystine / metabolism* Cystinosis / metabolism* Ethylmaleimide / pharmacology Humans Kinetics Leukocytes / metabolism* Lysosomes / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Carrier Proteins; 128-53-0/Ethylmaleimide; 52-90-4/Cysteine; 56-89-3/Cystine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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