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.
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.
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.
