Document Detail

Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis.
MedLine Citation:
PMID:  7112129     Owner:  NLM     Status:  MEDLINE    
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.
W A Gahl; N Bashan; F Tietze; I Bernardini; J D Schulman
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Science (New York, N.Y.)     Volume:  217     ISSN:  0036-8075     ISO Abbreviation:  Science     Publication Date:  1982 Sep 
Date Detail:
Created Date:  1982-10-21     Completed Date:  1982-10-21     Revised Date:  2007-03-19    
Medline Journal Info:
Nlm Unique ID:  0404511     Medline TA:  Science     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1263-5     Citation Subset:  IM    
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MeSH Terms
Biological Transport / drug effects
Carrier Proteins / metabolism
Cysteine / metabolism
Cystine / metabolism*
Cystinosis / metabolism*
Ethylmaleimide / pharmacology
Leukocytes / metabolism*
Lysosomes / metabolism
Reg. No./Substance:
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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