Tiopronin
Tiopronin is a reducing agent used to treat cystinuria, a genetic condition that leads to the formation of cystine kidney stones. It works by converting cystine into a water-soluble form that can be easily excreted in urine, thereby preventing stone formation.
Tiopronin prevents cystine kidney stones by reducing urinary cystine concentration through thiol-disulfide exchange.
Key facts at a glance
- ATC
- G04BX16
- RxNorm
- 6765
- Generic
- Tiopronin
- Class
- atc-g04
- Brands
- Thiola, Venxxiva
- Availability
- rx
- Lactation
- unknown
- Routes
- oral
- Forms
- tablet
- Regions
- US, EU, CN, JP
- Monitoring
- —
This page is for informational purposes only.
Do not use this information to diagnose yourself or to replace a physician's advice. Always consult a qualified healthcare provider before starting, stopping, or changing any medication. If you believe you are experiencing a medical emergency, call your local emergency number immediately.
Cystinuria, a hereditary condition causing cystine kidney stones due to impaired renal cystine reabsorption. The goal is to maintain urinary cystine concentration below its solubility limit to prevent stone formation.
Tiopronin is an active reducing agent that undergoes thiol-disulfide exchange with cystine, forming a water-soluble mixed disulfide (tiopronin-cysteine complex) that can be readily excreted in urine.
Requires regular monitoring of urinary cystine levels. May cause gastrointestinal side effects, skin reactions, or proteinuria. Contraindicated in patients with renal insufficiency. Should be used as part of a comprehensive management plan including hydration therapy and dietary modifications.
Official drug label
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This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed July 26, 2026. · Read our editorial policy