Article
Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines.
Biochemical and biophysical research communications - 5 Nov 2004
Chol Marie, Nevo Nathalie, Cherqui Stéphanie, Antignac Corinne, Rustin Pierre
Abstract excerpt
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 mechanis...
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