The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Cystine Transport Is Defective in Isolated Leukocyte Lysosomes from Patients with Cystinosis
Abstract
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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Cites
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
Thoene, J. G., Oshima, R. G., Crawhall, J. C., Olson, D. L., & Schneider, J. A. (1976) · Journal of Clinical Investigation · doi:10.1172/jci108448 · Cited by 336
Binding Assays for Amino Acids
Oshima, R. G., Willis, R. C., Furlong, C. E., & Schneider, J. A. (1974) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)42214-x · Cited by 155
Accumulation of amino acids by lysosomes incubated with amino acid methyl esters.
Reeves, J. P. (1979) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)86787-x · Cited by 135
Rupture of rat liver lysosomes mediated by l-amino acid esters
Goldman, R., & Kaplan, A. (1973) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/0005-2736(73)90114-4 · Cited by 108
Cystine accumulation and loss in normal, heterozygous, and cystinotic fibroblasts.
Jonas, A. J., Greene, A. A., Smith, M. L., & Schneider, J. A. (1982) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.79.14.4442 · Cited by 58
Degradation of mucopolysaccharide in intact isolated lysosomes.
Rome, L. H., & Crain, L. R. (1981) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)68506-6 · Cited by 55
Steinherz, R., Tietze, F., Gahl, W. A., et al. (1982) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.79.14.4446 · Cited by 52
Self-citation
Cited by
Saito, G., Swanson, J. A., & Lee, K.-D. (2003) · Advanced Drug Delivery Reviews · doi:10.1016/s0169-409x(02)00179-5 · Cited by 1,407
A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis
Town, M., Jean, G., Cherqui, S., Attard, M., Forestier, L., Whitmore, S. A., Callen, D. F., Gribouval, O., Broyer, M., Bates, G. P., et al. (1998) · Nature Genetics · doi:10.1038/ng0498-319 · Cited by 639
Hirschberg, C. B., Robbins, P. W., & Abeijon, C. (1998) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.67.1.49 · Cited by 386
Cystinosin, the protein defective in cystinosis, is a H+-driven lysosomal cystine transporter
Kalatzis, V. (2001) · The EMBO Journal · doi:10.1093/emboj/20.21.5940 · Cited by 331
Transport of cystine and cysteine in mammalian cells
Bannai, S. (1984) · Biochimica et Biophysica Acta (BBA) - Reviews on Biomembranes · doi:10.1016/0304-4157(84)90014-5 · Cited by 329
Metadata
- Authors
- Gahl, W. A., Bashan, N., Tietze, F., Bernardini, I., & Schulman, J. D.
- Venue
- Science
- Published
- 1982-09-24
- Volume
- 217
- Issue
- 4566
- Pages
- 1263-1265
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W1975360166
- Cited by
- 360
- References
- 7
- FWCI
- 9.61
- Type
- journal article
- Language
- en
- Open access
- closed
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- Gahl, W. A., Bashan, N., Tietze, F., Bernardini, I., & Schulman, J. D. (1982). Cystine Transport Is Defective in Isolated Leukocyte Lysosomes from Patients with Cystinosis. Science. https://doi.org/10.1126/science.7112129
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
