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. 1982 Jul;79(14):4446–4450. doi: 10.1073/pnas.79.14.4446

Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester.

R Steinherz, F Tietze, W A Gahl, T J Triche, H Chiang, A Modesti, J D Schulman
PMCID: PMC346689  PMID: 6956873

Abstract

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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