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Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.
Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.
- Source :
-
Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2004 Nov 05; Vol. 324 (1), pp. 231-5. - Publication Year :
- 2004
-
Abstract
- Cystinosis is an inherited disorder due to mutations in the CTNS gene which encodes cystinosin, a lysosomal transmembrane protein involved in cystine export to the cytosol. Both accumulation of cystine in the lysosome and decreased cystine in the cytosol may participate in the pathogenic mechanism underlying the disease. We observed that cystinotic cell lines have moderate decrease of glutathione content during exponential growth phase. This resulted in increased solicitation of oxidative defences of the cell denoted by concurrent superoxide dismutase induction, although without major oxidative insult under our experimental conditions. Finally, decreased glutathione content in cystinotic cell lines could be counterbalanced by a series of exogenous precursors of cysteine, denoting that lysosomal cystine export is a natural source of cellular cysteine in the studied cell lines.
- Subjects :
- Adolescent
Amino Acid Transport Systems, Neutral
Animals
Cell Line
Child
Cystine metabolism
Cystinosis genetics
Cystinosis pathology
Fibroblasts cytology
Fibroblasts metabolism
Glycoproteins genetics
Glycoproteins metabolism
Humans
Infant
Membrane Proteins genetics
Membrane Proteins metabolism
Membrane Transport Proteins
Mutation
Oxidation-Reduction
Superoxide Dismutase metabolism
Cystinosis metabolism
Glutathione metabolism
Protein Precursors metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0006-291X
- Volume :
- 324
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Biochemical and biophysical research communications
- Publication Type :
- Academic Journal
- Accession number :
- 15465007
- Full Text :
- https://doi.org/10.1016/j.bbrc.2004.09.033