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Tiopronin is a thiol agent indicated for the prevention of kidney stone formation in patients with severe homozygous cystinuria. Patients with cystinuria excrete high levels of cystine in their urine and are at risk for kidney stone formation.
- Mechanism
- Curator reviewed
Kidney stones form when the solubility limit is exceeded and urine becomes supersaturated with endogenous cystine. Tiopronin is an active reducing agent which undergoes a thiol-disulfide exchange with cystine to form a water-soluble mixed disulfide complex. Thus, the amount of sparingly soluble cystine is reduced. By reducing urinary cystine concentrations below the solubility limit, tiopronin helps reduce cystine stone formation.
- Primary indication
- Tiopronin is indicated for the prevention of kidney stone formation in patients with severe homozygous cystinuria consisting of a urinary cystine concentration greater than 500 mg/day, and who have failed treatment with non-pharmacological measures of...Curator reviewed · 1 structured indication
- Formula / weight
- C5H9NO3S · 163.19 g/mol (avg)
- First approval
- United States, 1988
- Brand names
- Thiola
- Venxxiva
Resolves to
YTGJWQPHMWSCST-UHFFFAOYSA-NSMILESCC(S)C(=O)NCC(O)=OWhat you can answer from here — as of July 03, 2026
Which drugs share a target with Tiopronin, and which of those have an active Phase 3 trial?