Stereochemistry | RACEMIC |
Molecular Formula | C5H9NO3S |
Molecular Weight | 163.195 |
Optical Activity | ( + / - ) |
Defined Stereocenters | 0 / 1 |
E/Z Centers | 0 |
Charge | 0 |
SHOW SMILES / InChI
SMILES
CC(S)C(=O)NCC(O)=O
InChI
InChIKey=YTGJWQPHMWSCST-UHFFFAOYSA-N
InChI=1S/C5H9NO3S/c1-3(10)5(9)6-2-4(7)8/h3,10H,2H2,1H3,(H,6,9)(H,7,8)
Molecular Formula | C5H9NO3S |
Molecular Weight | 163.195 |
Charge | 0 |
Count |
MOL RATIO
1 MOL RATIO (average) |
Stereochemistry | RACEMIC |
Additional Stereochemistry | No |
Defined Stereocenters | 0 / 1 |
E/Z Centers | 0 |
Optical Activity | ( + / - ) |
Tiopronin is a prescription thiol drug used primarily in the treatment of severe homozygous cystinuria. Patients with cystinuria excrete high levels of cystine in their urine and are at risk for kidney stone formation. Tiopronin is used as a second-line therapy to control the rate of cystine precipitation and excretion, and prevent kidney stone formation. It is used after a failure of the non-pharmacological first line treatment consisting of increased fluid intake, restriction of sodium and protein, and urinary alkalinization. As cystinuria is a relatively rare disease, tiopronin is classified as an orphan drug and is not patented in the United States. It is similar to d-penicillamine in use and efficacy, but offers the advantage of far less adverse effects. Tiopronin is dosed on an individual basis using close monitoring of urinary cystine concentrations and urinary output. Tiopronin is a chelating agent. It works by removing extra cystine (the cause of kidney stones) from the urine, which keeps the kidney stones from forming. It works by reacting with urinary cysteine to form a more soluble, disulfide linked, tiopronin-cysteine complex.
Originator
Approval Year
Doses
AEs
Overview
CYP3A4 | CYP2C9 | CYP2D6 | hERG |
---|---|---|---|
OverviewOther
Other Inhibitor | Other Substrate | Other Inducer |
---|---|---|
Drug as perpetrator
Drug as victim
Sourcing
PubMed
Patents
Sample Use Guides
THIOLA (Tiopronin) may be begun at a dosage of 800 mg/day in adult patients with cystine stones. In a multiclinic trial, average dose of THIOLA was about 1000 mg/day. In children, initial dosage may be based on 15 mg/kg/day. Whenever possible, THIOLA should be given in divided doses 3 times/day at least one hour before or 2 hours after meals.
Route of Administration:
Oral
Tiopronin (2 mM) completely prevents the Cisplatin-induced increase in enzyme leakage and substantially blocks the decrease of 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) reduction caused by Cisplatin. Tiopronin also significantly protects the renal slices from cisplatin-induced toxic effects. Tiopronin protects against cisplatin-induced nephrotoxicity by acting as an alternative target for Cisplatin both intra- and extracellularly and thus protects against cisplatin-induced depletion of glutathione in the kidney cell