tiopronin

Tiopronin is a small molecule thiol drug used primarily to prevent kidney stone formation in patients with severe homozygous cystinuria who are not responsive to non-pharmacological measures such as increased fluid intake, dietary modification, and urinary alkalinization. It acts as an active reducing agent that undergoes thiol-disulfide exchange with cystine in the urine to form a water-soluble mixed disulfide complex (tiopronin-cysteine), thereby reducing the concentration of sparingly soluble cystine and preventing its precipitation into stones. Tiopronin is considered a second-line therapy after failure of conservative management and is classified as an orphan drug due to the rarity of cystinuria[1][2][5][6].

Brand names

ThiolaThiola ECVenxxiva

Other names

2-mercaptopropionylglycine