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Boost in cystine transporter action following CystagonR administration observed within 6-9 hours. Implications on cystinosin cycle and cellular health.
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cystinosin cytosole Cysteine –cysteamine cystine cysteamine cysteine NH2 COOH CH CH2 S S CH2 CH NH2 COOH NH2 COOH CH CH2 S S CH2 CH2 NH2 SH CH2 CH NH2 COOH HS CH2 CH2 NH2 lysosome + + lysine transporter Cysteine transporter
cystine nmol/mg protein p < 0.05 6 hours after CystagonR dose 9 hours after CystagonR dose