Summary
Cystinuria, an inborn error of metabolism, is clinically characterized by the formation of l-cystine crystals and stones in the urinary tract. Sulfhydryl compounds react with the less soluble cystine to form water-soluble disulfide derivatives. l-Cystine stone formation and its undesirable side effects are thus prevented, and regulation of its metabolism results. The mechanism of action for the dissolution of l-cystine is based on a Thiol-Disulfide exchange reaction. The dissolving capacity of the sulfhydryl compounds can be enhanced by coupling them with amino acids, for example the mercaptopeptides, glutathion and Thiola. A further enhancement of the dissolving capacity of the sulfhydryl drugs may be achieved by adding a second sulfhydryl group to their molecule, resulting in a Dithiol-Disulfide exchange reaction, which accelerates cystine dissolution.
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Abbreviations and synonyms for Thiola: (a-MPG), (MPG), a-mer-capto-propionyl-glycine and N-(2-mercaptopropionyl)glycine.
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Kallistratos, G., Kalfakakou-Vadalouka, V., Evangelou, A. et al. Sulfhydryl compounds in the treatment of cystinuria. World J Urol 4, 122–126 (1986). https://doi.org/10.1007/BF00326406
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DOI: https://doi.org/10.1007/BF00326406