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Details

Stereochemistry RACEMIC
Molecular Formula C5H9NO3S
Molecular Weight 163.195
Optical Activity ( + / - )
Defined Stereocenters 0 / 1
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of TIOPRONIN

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)

HIDE SMILES / InChI

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 ( + / - )

Description

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

Targets

Primary TargetPharmacologyConditionPotency

Conditions

ConditionModalityTargetsHighest PhaseProduct
Primary
THIOLA

Cmax

ValueDoseCo-administeredAnalytePopulation
3.6 μg/mL
500 mg single, oral
TIOPRONIN plasma
Homo sapiens

AUC

ValueDoseCo-administeredAnalytePopulation
29 μg × h/mL
500 mg single, oral
TIOPRONIN plasma
Homo sapiens

T1/2

ValueDoseCo-administeredAnalytePopulation
18.7 h
500 mg single, oral
TIOPRONIN plasma
Homo sapiens

Doses

AEs

Overview

CYP3A4CYP2C9CYP2D6hERG

OverviewOther

Other InhibitorOther SubstrateOther Inducer

Drug as perpetrator​

Drug as victim

PubMed

Patents

Sample Use Guides

In Vivo Use Guide
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
In Vitro Use Guide
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
Substance Class Chemical
Record UNII
C5W04GO61S
Record Status Validated (UNII)
Record Version