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Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.

Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.

Authors :
Chol M
Nevo N
Cherqui S
Antignac C
Rustin P
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.

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