Description of a selection method highly cytotoxic for cystinotic fibroblasts but not normal human fibroblasts
dc.contributor.author | Lemons, Rosemary M. | en_US |
dc.contributor.author | Thoene, Jess G. | en_US |
dc.contributor.author | Pisoni, Ronald L. | en_US |
dc.contributor.author | Paelicke, Karen M. | en_US |
dc.date.accessioned | 2006-09-11T16:10:56Z | |
dc.date.available | 2006-09-11T16:10:56Z | |
dc.date.issued | 1992-01 | en_US |
dc.identifier.citation | Pisoni, Ronald L.; Lemons, Rosemary M.; Paelicke, Karen M.; Thoene, Jess G.; (1992). "Description of a selection method highly cytotoxic for cystinotic fibroblasts but not normal human fibroblasts." Somatic Cell and Molecular Genetics 18(1): 1-6. <http://hdl.handle.net/2027.42/45541> | en_US |
dc.identifier.issn | 1572-9931 | en_US |
dc.identifier.issn | 0740-7750 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/45541 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=1546366&dopt=citation | en_US |
dc.description.abstract | Nephropathic cystinosis is an inherited disorder characterized by a high intralysosomal accumulation of cystine due to a defect in lysosomal cystine transport. Cystine can be specifically loaded into the lysosomal compartment of intact cells by incubating cells with cystine dimethyl ester (CDME). We have applied this methyl ester loading technique to develop a selection method that is highly cytotoxic for cystinotic fibroblasts but not normal human fibroblasts and that is based on the inherent differences in lysosomal cystine transport activity of normal and cystinotic fibroblasts. Thus, only 0–0.03% of fetal cystinotic fibroblasts survive exposure to 2 mM CDME for 20 min whereas 70–80% of normal fetal fibroblasts survive these same conditions. Following transfection of cystinotic fibroblasts with normal human genomic DNA or cDNA, this CDME selection method can be used to select for those cells that have been transformed to the normal phenotype and thus aid in the identification of the gene coding for the lysosomal cystine transport protein. | en_US |
dc.format.extent | 611288 bytes | |
dc.format.extent | 3115 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.language.iso | en_US | |
dc.publisher | Kluwer Academic Publishers-Plenum Publishers; Plenum Publishing Corporation ; Springer Science+Business Media | en_US |
dc.subject.other | Plant Sciences | en_US |
dc.subject.other | Human Genetics | en_US |
dc.subject.other | Biomedicine | en_US |
dc.subject.other | Biochemistry, General | en_US |
dc.subject.other | Animal Anatomy / Morphology / Histology | en_US |
dc.title | Description of a selection method highly cytotoxic for cystinotic fibroblasts but not normal human fibroblasts | en_US |
dc.type | Article | en_US |
dc.subject.hlbsecondlevel | Natural Resources and Environment | en_US |
dc.subject.hlbsecondlevel | Molecular, Cellular and Developmental Biology | en_US |
dc.subject.hlbsecondlevel | Ecology and Evolutionary Biology | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.subject.hlbtoplevel | Science | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Department of Pediatrics, The University of Michigan Medical School, 48109-2029, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationum | Department of Pediatrics, The University of Michigan Medical School, 48109-2029, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationum | Department of Pediatrics, The University of Michigan Medical School, 48109-2029, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationum | Department of Pediatrics, The University of Michigan Medical School, 48109-2029, Ann Arbor, Michigan; Department of Biological Chemistry, The University of Michigan Medical School, 48109-2029, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationumcampus | Ann Arbor | en_US |
dc.identifier.pmid | 1546366 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/45541/1/11188_2005_Article_BF01233444.pdf | en_US |
dc.identifier.doi | http://dx.doi.org/10.1007/BF01233444 | en_US |
dc.identifier.source | Somatic Cell and Molecular Genetics | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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