文章基本信息

标题：Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester
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作者：R Steinherz ; F Tietze ; W A Gahl 等
期刊名称：Proceedings of the National Academy of Sciences
印刷版ISSN：0027-8424
电子版ISSN：1091-6490
出版年度：1982
卷号：79
期号：14
页码：4446-4450
DOI：10.1073/pnas.79.14.4446
语种：English
出版社：The National Academy of Sciences of the United States of America
摘要：Upon exposure to 0.25 mM cystine dimethyl ester, normal and cystinotic leukocytes accumulate substantially more intracellular cystine than is present endogenously in cystinotic cells. Leukocytes loaded by exposure to cystine dimethyl ester may have abnormally lucent and distended lysosomes, and the cystine is compartmentalized within the granular fraction of the cells. After the cells are exposed to cystine dimethyl ester, cystine clearance from normal leukocytes is much faster than from cystinotic cells. The ratios of labeled cysteine-N-ethylmaleimide to cystine are also greater in normals than in cystinotics 60 min after termination of loading. No overlap in ranges of cystine clearance half-times or cystine-N-ethylmaleimide to cystine ratios was observed in normal compared to cystinotic leukocytes. Limited experiments with fibroblasts exposed to cystine dimethyl ester suggest a correspondingly prolonged cystine clearance for cystinotic cells. These experiments provide evidence for defective clearance of cystine from cystinotic lysosomes in situ.