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cystinosin

cystinosin. cytosole. Cysteine –cysteamine. cystine. cysteamine. cysteine. NH 2 COOH CH CH 2 S S CH 2 CH NH 2 COOH. NH 2 COOH CH CH 2 S S CH 2

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cystinosin

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  1. 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

  2. cystine nmol/mg protein p < 0.05 6 hours after CystagonR dose 9 hours after CystagonR dose

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