Document Detail

Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.
MedLine Citation:
PMID:  15465007     Owner:  NLM     Status:  MEDLINE    
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.
Marie Chol; Nathalie Nevo; Stéphanie Cherqui; Corinne Antignac; Pierre Rustin
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Biochemical and biophysical research communications     Volume:  324     ISSN:  0006-291X     ISO Abbreviation:  Biochem. Biophys. Res. Commun.     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-10-06     Completed Date:  2004-12-28     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0372516     Medline TA:  Biochem Biophys Res Commun     Country:  United States    
Other Details:
Languages:  eng     Pagination:  231-5     Citation Subset:  IM    
INSERM U574, Néphropathies Héréditaires et Rein en Développement, Hôpital Necker-Enfants Malades, rue de Sèvres, 75015 Paris, France.
Data Bank Information
Bank Name/Acc. No.:
OMIM/219750;  219800;  219900
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MeSH Terms
Amino Acid Transport Systems, Neutral
Cell Line
Cystine / metabolism
Cystinosis / genetics,  metabolism*,  pathology
Fibroblasts / cytology,  metabolism
Glutathione / metabolism*
Glycoproteins / genetics,  metabolism
Membrane Proteins / genetics,  metabolism
Membrane Transport Proteins
Protein Precursors / metabolism*
Superoxide Dismutase / metabolism
Reg. No./Substance:
0/Amino Acid Transport Systems, Neutral; 0/CTNS protein, human; 0/Glycoproteins; 0/Membrane Proteins; 0/Membrane Transport Proteins; 0/Protein Precursors; 56-89-3/Cystine; 70-18-8/Glutathione; EC Dismutase

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