Publication Date:
1982-09-24
Description:
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Gahl, W A -- Bashan, N -- Tietze, F -- Bernardini, I -- Schulman, J D -- New York, N.Y. -- Science. 1982 Sep 24;217(4566):1263-5.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/7112129" target="_blank"〉PubMed〈/a〉
Keywords:
Biological Transport/drug effects
;
Carrier Proteins/metabolism
;
Cysteine/metabolism
;
Cystine/*metabolism
;
Cystinosis/*metabolism
;
Ethylmaleimide/pharmacology
;
Humans
;
Kinetics
;
Leukocytes/*metabolism
;
Lysosomes/metabolism
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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