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Figure Caption
Figure 7 A hypothetical model illustrating the effects of DSF in cystinotic cells. DSF (DDC-S-S-DDC) is a disulfide that is reduced to diethyldithiocarbamate (DDC) in the cytosol, consuming GSH and other free thiols. Consequently, cells are more exposed to free radicals, causing oxidative cell damage and death. Similarly to cysteamine, reduced DDC can react with cystine (C-SS-C) in lysosomes, forming a mixed disulfide (DDC-S-S-C) that allows clearance of cystine.
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