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CYSTEAMINE HYDROCHLORIDE for synthesis

CYSTEAMINE HYDROCHLORIDE for synthesis
SKU
RXSOL-60-6604-395
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Synthesis of sup 14/C-cysteamine and cystamine. Bulgaria. The cysteamine hydrochloride compound was obtained by dissolving 100 mg of the cysteamine salicylate in 5 ml of water, acidifying with concentrated hydrochloric acid to a pH of 1 to 2, and extracting with 5 ml of ether five times. Yield is 90 percent.

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Cysteamine, as a part of CoA plays a crucial role in metabolism by facilitating thioester formation. However, as a free entity, cysteamine is largely absent from metabolism. By contrast, supraphysiological amounts of this simple aminothiol have profound and selective effects of physiology and certain diseases. 


Cysteamine is a cystine depleting agent used to treat the effects of cystinosis. Cystinosis is a rare disease caused by mutations in the CTNS gene that encodes for cystinosin, a protein responsible for transporting cystine out of the cell lysosome.


110 lbs or more:
-Initial dose: 0.3 to 0.5 g/day orally divided in 4 doses. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 2 nmol half-cystine/mg protein.
-Maintenance dose: 2 g/day orally divided in 4 doses
 
Delayed-release capsule:
-Initial dose: 0.2 to 0.3 g/m2/day orally divided in 2 doses, every 12 hours. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 1 nmol half-cystine/mg protein.
-Maintenance dose: 1.3 g/m2/day orally divided in 2 doses, every 12 hours
Maximum dose: 1.95 g/m2/day
-Maximum dose: 1.95 g/m2/day110 lbs or more:
-Initial dose: 0.3 to 0.5 g/day orally divided in 4 doses. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 2 nmol half-cystine/mg protein.
-Maintenance dose: 2 g/day orally divided in 4 doses
 
Delayed-release capsule:
-Initial dose: 0.2 to 0.3 g/m2/day orally divided in 2 doses, every 12 hours. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 1 nmol half-cystine/mg protein.
-Maintenance dose: 1.3 g/m2/day orally divided in 2 doses, every 12 hours
-Maximum dose: 1.95 g/m2/day


 

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