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Review

Improving the prognosis of nephropathic cystinosis

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Pages 297-302 | Published online: 17 Jul 2014

Figures & data

Figure 1 Mechanism of lysosomal cystine depletion by cysteamine. Cysteamine enters the lysosome through an unknown transporter and breaks the disulfide bond in cystine. This results in formation of cysteine and a new cysteine-cysteamine mixed disulfide, each of which can exit the lysosome through its own transporter.

Figure 1 Mechanism of lysosomal cystine depletion by cysteamine. Cysteamine enters the lysosome through an unknown transporter and breaks the disulfide bond in cystine. This results in formation of cysteine and a new cysteine-cysteamine mixed disulfide, each of which can exit the lysosome through its own transporter.