U.S. Department of Health & Human Services Divider Arrow National Institutes of Health Divider Arrow NCATS

Details

Stereochemistry EPIMERIC
Molecular Formula C6H14N4O2.C5H9NO3S
Molecular Weight 337.396
Optical Activity UNSPECIFIED
Defined Stereocenters 1 / 2
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of TIOPRONIN ARGININE

SMILES

CC(S)C(=O)NCC(O)=O.N[C@@H](CCCNC(N)=N)C(O)=O

InChI

InChIKey=CFAXANACVDYTPS-WCCKRBBISA-N
InChI=1S/C6H14N4O2.C5H9NO3S/c7-4(5(11)12)2-1-3-10-6(8)9;1-3(10)5(9)6-2-4(7)8/h4H,1-3,7H2,(H,11,12)(H4,8,9,10);3,10H,2H2,1H3,(H,6,9)(H,7,8)/t4-;/m0./s1

HIDE SMILES / InChI
Molecular Formula C5H9NO3S
Molecular Weight 163.195
Charge 0
Count
Stereochemistry RACEMIC
Additional Stereochemistry No
Defined Stereocenters 0 / 1
E/Z Centers 0
Optical Activity ( + / - )
Molecular Formula C6H14N4O2
Molecular Weight 174.201
Charge 0
Count
Stereochemistry ABSOLUTE
Additional Stereochemistry No
Defined Stereocenters 1 / 1
E/Z Centers 0
Optical Activity UNSPECIFIED

Description
Curator's Comment: description was created based on several sources, including: http://www.drugbank.ca/drugs/DB06823 https://en.wikipedia.org/wiki/Tiopronin

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

1988
90
Targets

Targets

Primary TargetPharmacologyConditionPotency
Chelating Agent
139
Conditions

Conditions

ConditionModalityTargetsHighest PhaseProduct
Primary
Approved
8583
THIOLA

Approved Use

Tiopronin

Launch Date

1988
Cmax

Cmax

ValueDoseCo-administeredAnalytePopulation
3.6 μg/mL
EXPERIMENT
https://www.ncbi.nlm.nih.gov/pubmed/1505618
500 mg single, oral
dose: 500 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
TIOPRONIN plasma
Homo sapiens
population: HEALTHY
age: ADULT
sex: MALE
food status: UNKNOWN
AUC

AUC

ValueDoseCo-administeredAnalytePopulation
29 μg × h/mL
EXPERIMENT
https://www.ncbi.nlm.nih.gov/pubmed/1505618
500 mg single, oral
dose: 500 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
TIOPRONIN plasma
Homo sapiens
population: HEALTHY
age: ADULT
sex: MALE
food status: UNKNOWN
T1/2

T1/2

ValueDoseCo-administeredAnalytePopulation
18.7 h
EXPERIMENT
https://www.ncbi.nlm.nih.gov/pubmed/1505618
500 mg single, oral
dose: 500 mg
route of administration: Oral
experiment type: SINGLE
co-administered:
TIOPRONIN plasma
Homo sapiens
population: HEALTHY
age: ADULT
sex: MALE
food status: UNKNOWN
Doses

Doses

DosePopulationAdverse events​
1 g 3 times / day multiple, oral
Highest studied dose
Dose: 1 g, 3 times / day
Route: oral
Route: multiple
Dose: 1 g, 3 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/20559104/
unhealthy, 43-74
Health Status: unhealthy
Age Group: 43-74
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/20559104/
Sources:
https://pubmed.ncbi.nlm.nih.gov/20559104/
1 g 1 times / day multiple, oral
Highest studied dose
Dose: 1 g, 1 times / day
Route: oral
Route: multiple
Dose: 1 g, 1 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
unhealthy, 56.7
Health Status: unhealthy
Age Group: 56.7
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
Other AEs: Proteinuria, Stomatitis...
Other AEs:
Proteinuria (4 patients)
Stomatitis (3 patients)
Itching (2 patients)
Discomfort epigastric (1 patient)
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Other AEs: Anemia, Nausea...
Other AEs:
Anemia (6%)
Nausea (12%)
Emesis
Diarrhea (6%)
Abdominal pain (6%)
Oral ulceration (18%)
Fever
Weakness (12%)
Fatigue
Edema peripheral (6%)
Chest pain (6%)
Anorexia
Arthralgia (12%)
Proteinuria (6%)
Impotence (6%)
Cough (6%)
Rash (12%)
Ecchymosis
Pruritus (6%)
Urticaria
Skin wrinkling (6%)
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Other AEs: Anemia, Nausea...
Other AEs:
Anemia (2%)
Nausea (25%)
Emesis (10%)
Diarrhea (18%)
Abdominal pain
Oral ulceration (12%)
Fever (8%)
Weakness (4%)
Fatigue (14%)
Edema peripheral (6%)
Chest pain
Anorexia (8%)
Arthralgia
Proteinuria (10%)
Impotence
Cough
Rash (14%)
Ecchymosis (6%)
Pruritus (4%)
Urticaria (8%)
Skin wrinkling (6%)
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
AEs

AEs

AESignificanceDosePopulation
Discomfort epigastric 1 patient
1 g 1 times / day multiple, oral
Highest studied dose
Dose: 1 g, 1 times / day
Route: oral
Route: multiple
Dose: 1 g, 1 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
unhealthy, 56.7
Health Status: unhealthy
Age Group: 56.7
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
Itching 2 patients
1 g 1 times / day multiple, oral
Highest studied dose
Dose: 1 g, 1 times / day
Route: oral
Route: multiple
Dose: 1 g, 1 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
unhealthy, 56.7
Health Status: unhealthy
Age Group: 56.7
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
Stomatitis 3 patients
1 g 1 times / day multiple, oral
Highest studied dose
Dose: 1 g, 1 times / day
Route: oral
Route: multiple
Dose: 1 g, 1 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
unhealthy, 56.7
Health Status: unhealthy
Age Group: 56.7
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
Proteinuria 4 patients
1 g 1 times / day multiple, oral
Highest studied dose
Dose: 1 g, 1 times / day
Route: oral
Route: multiple
Dose: 1 g, 1 times / day
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
unhealthy, 56.7
Health Status: unhealthy
Age Group: 56.7
Sex: M+F
Sources:
https://pubmed.ncbi.nlm.nih.gov/7115451/
Anorexia
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Ecchymosis
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Emesis
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Fatigue
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Fever
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Urticaria
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Arthralgia 12%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Nausea 12%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Rash 12%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Weakness 12%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Oral ulceration 18%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Abdominal pain 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Anemia 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Chest pain 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Cough 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Diarrhea 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Edema peripheral 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Impotence 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Proteinuria 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Pruritus 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Skin wrinkling 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Abdominal pain
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Arthralgia
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Chest pain
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Cough
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Impotence
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Emesis 10%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Proteinuria 10%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Oral ulceration 12%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Fatigue 14%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Rash 14%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Diarrhea 18%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Anemia 2%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Nausea 25%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Pruritus 4%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Weakness 4%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Ecchymosis 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Edema peripheral 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Skin wrinkling 6%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Anorexia 8%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Fever 8%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Urticaria 8%
0.267 g 3 times / day multiple, oral
Recommended
Dose: 0.267 g, 3 times / day
Route: oral
Route: multiple
Dose: 0.267 g, 3 times / day
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
unhealthy
Health Status: unhealthy
Sources:
https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/211843s000lbl.pdf
Overview

Overview

CYP3A4CYP2C9CYP2D6hERG

OverviewOther

Other InhibitorOther SubstrateOther Inducer
OAT3
391
Drug as perpetrator​

Drug as perpetrator​

TargetModalityActivityMetaboliteClinical evidence
MRP1
232
 yes
P-gp
1932
 yes
Drug as victim

Drug as victim

TargetModalityActivityMetaboliteClinical evidence
OAT3
391
 yes
Sourcing

Sourcing

Vendor/AggregatorIDURL
001 Chemical
DY536622
https://www.001chemical.com/chem/1953-02-2
3B Scientific (Wuhan) Corp
3B2-0198
http://www.3bsc.com/index/pro_info.php?id=19554
AA Blocks
AA0035MI
https://www.aablocks.com/goods/Goods/detail?id=AA0035MI
Aaron Chemicals
AR0036EA
http://www.aaronchem.com/1953-02-2
abcr GmbH
AB261737
http://www.abcr.com/shop/en/AB261737
AbMole Bioscience
M6015
http://www.abmole.com/products/tiopronin.html
Acadechem
ACDS-027577
http://www.acadechemical.com/product/102276
Accela ChemBio Inc
SY262996
http://www.accelachem.com/cn/search.html?keyword=SY262996&attname=GoodsCode
Achemo Scientific Limited
AC-11491
http://www.achemo.com/product_view.asp?ID=1381
AHH Chemical co.,ltd
MT-59530
http://www.ahhchemical.com/product/MT-59530.html
AK Scientific, Inc. (AKSCI)
70454
http://www.aksci.com/item_detail.php?cat=70454
Allbio Pharm Co., Ltd
AD08526
http://www.allbiopharm.com/product/n/AD08526
Angene
AGN-PC-07A27E
http://www.angenechemical.com/productshow/AGN-PC-07A27E.html
Anward
ANW-41587
http://www.anward.com/pro_result/26370
Ark Pharm
AK501603
http://www.arkpharminc.com/products/1953-02-2.html
BioChemPartner
BCP13354
http://www.biochempartner.com/1953-02-2-BCP13354
BioCrick
BCC3870
http://www.biocrick.com/Tiopronin-Thiola-BCC3870.html
BioSynth
J-012651
https://www.biosynth.com/en/products/all-products/products/J-012651.html
BLD Pharm
BD2874
http://www.bldpharm.com/products/1953-02-2.html
Capot Chemical
15029
http://www.capotchem.com/en/15029.htm
Capot Chemical
PubChem15029
http://www.capotchem.com/en/15029.htm
Chem Scene
CS-2431
http://www.chemscene.com/1953-02-2.html
ChemFish Tokyo co.,ltd
5483
http://www.chemfish.co.jp/index.php?id=4620&project=product
ChemMol
30104676
http://www.chemmol.com/chemmol/30104676.html
ChemMol
49408236
http://www.chemmol.com/chemmol/49408236.html
Chemspace
CSSB00000031781
https://chem-space.com/CSSB00000031781
CSNpharm
CSN13774
https://www.csnpharm.com/products/tiopronin.html
DC Chemicals
DCAPI1105
http://www.dcchemicals.com//product_show-Tiopronin_Thiola_.html
eNovation Chemicals
D671098
https://www.enovationchem.com/ProductDetails.asp?ProductID=D671098
Finetech
FT-0603530
http://www.finetechnology-ind.com/prod/detail/pub/1953-02-2.html
Finetech
FT-0653686
http://www.finetechnology-ind.com/prod/detail/pub/1953-02-2.html
Glentham Life Sciences
GM4146
http://www.glentham.com/products/product/GM4146/
Hairui
HR137424
http://www.hairuichem.com/en/product/1953-02-2.html
Lab Network
LN01300535
https://labnetwork.com/frontend-app/p/#!/moleculedetails/LN01300535
LGC Standards
DRE-C17587000
https://www.lgcstandards.com/US/en/p/DRE-C17587000
mcule
MCULE-8593628629
https://mcule.com/MCULE-8593628629/
MedChem Express
HY-B0373
https://www.medchemexpress.com/Tiopronin.html
Molcore
MC536622
https://www.molcore.com/product/1953-02-2
MuseChem
A000514
https://www.musechem.com/product/A000514.html
Oakwood
99155
http://www.oakwoodchemical.com/ProductsList.aspx?CategoryID=-2&txtSearch=162585&ExtHyperLink=1
OChem
5409
http://www.ocheminc.com/index.php?act=psearch&pid=953T022
Sigma-Aldrich
280968_ALDRICH
https://www.sigmaaldrich.com/catalog/product/aldrich/280968?utm_source=pubchem&utm_campaign=pubchem_2017&utm_medium=referral
Sigma-Aldrich
33986_SIAL
https://www.sigmaaldrich.com/catalog/product/sial/33986?utm_source=pubchem&utm_campaign=pubchem_2017&utm_medium=referral
Sigma-Aldrich
M6635_SIGMA
https://www.sigmaaldrich.com/catalog/product/sigma/m6635?utm_source=pubchem&utm_campaign=pubchem_2017&utm_medium=referral
Sinfoo Biotech
S011920
http://www.sinfoobiotech.com/product/1015318
Smolecule
S545402
http://www.smolecule.com/products/s545402
Smolecule
S793344
https://www.smolecule.com/products/s793344
TCI (Tokyo Chemical Industry)
T2614
http://www.tcichemicals.com/eshop/en/us/commodity/T2614/index.html
THE BioTek
bt-285718
https://www.thebiotek.com/product/inhibitors/bt-285718
Yuhao Chemical
SY0182
http://www.chemyuhao.com/1953-2-2.html
PubMed

PubMed

TitleDatePubMed
Effect of change in cellular GSH levels on mitochondrial damage and cell viability loss due to mitomycin c in small cell lung cancer cells.
2004-11-01
Nanoparticles comprising a mixed monolayer for specific bindings with biomolecules.
2004-09-29
Complexation of polysaccharide and monosaccharide with thiolate boronic acid capped on silver nanoparticle.
2004-09-15
HPLC study of tyrosinase inhibition by thiopronine.
2004-09
Transport of meprin subunits through the secretory pathway: role of the transmembrane and cytoplasmic domains and oligomerization.
2004-08-13
Liquid chromatography analysis of N-(2-mercaptopropionyl)-glycine in biological samples by ThioGlo 3 derivatization.
2004-08-05
Delayed ischemic preconditioning activates nuclear-encoded electron-transfer-chain gene expression in parallel with enhanced postanoxic mitochondrial respiratory recovery.
2004-08-03
Polyherbal extract of septilin protects mice against whole body lethal dose of gamma radiation.
2004-08
Yellow nail syndrome in rheumatoid arthritis: a drug-induced disease?
2004-08
Exogenous NO triggers preconditioning via a cGMP- and mitoKATP-dependent mechanism.
2004-08
Oligonucleotide-displaced organic monolayer-protected silver nanoparticles and enhanced luminescence of their salted aggregates.
2004-07-01
The MAM (meprin/A5-protein/PTPmu) domain is a homophilic binding site promoting the lateral dimerization of receptor-like protein-tyrosine phosphatase mu.
2004-06-25
Protective effects of alpha-tocopherol and tiopronin against cisplatin-induced ototoxicity.
2004-05
The role of antioxidants in protection from ototoxic drugs.
2004-05
Cardioprotection of interleukin-2 is mediated via kappa-opioid receptors.
2004-05
Exogenous nitric oxide generates ROS and induces cardioprotection: involvement of PKG, mitochondrial KATP channels, and ERK.
2004-04
The effects of ionizing radiation on DNA: the role of thiols as radioprotectors.
2004-03-26
Protective effect of N2-mercaptopropionylglycine on rats and dogs liver during ischemia/reperfusion process.
2004-03-19
Reactive oxygen species precede protein kinase C-delta activation independent of adenosine triphosphate-sensitive mitochondrial channel opening in sevoflurane-induced cardioprotection.
2004-03
ROS-mediated ERK activation in delayed protection from anoxic preconditioning in neonatal rat cardiomyocytes.
2004-03
Evidence of myofibrillar protein oxidation induced by postischemic reperfusion in isolated rat hearts.
2004-03
Remote preconditioning by infrarenal aortic occlusion is operative via delta1-opioid receptors and free radicals in vivo in the rat heart.
2004-02-15
Activation of mitochondrial ATP-sensitive potassium channels delays ischemia-induced cellular uncoupling in rat heart.
2004-01
Delayed cardioprotection is mediated via a non-peptide delta opioid agonist, SNC-121, independent of opioid receptor stimulation.
2004-01
Bradykinin induces mitochondrial ROS generation via NO, cGMP, PKG, and mitoKATP channel opening and leads to cardioprotection.
2004-01
Au nanoparticle-enhanced surface plasmon resonance sensing of biocatalytic transformations.
2003-12-15
A simple and sensitive chemiluminescence method for the determination of tiopronin for a pharmaceutical formulation.
2003-12-04
Studies of urinary cystine precipitation in vitro: ontogeny of cystine nephrolithiasis and identification of meso-2,3-dimercaptosuccinic acid as a potential therapy for cystinuria.
2003-12
Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds.
2003-12
Inhibition of endogenous thioredoxin in the heart increases oxidative stress and cardiac hypertrophy.
2003-11
The cardioprotective effect of sevoflurane depends on protein kinase C activation, opening of mitochondrial K(+)(ATP) channels, and the production of reactive oxygen species.
2003-11
Cardiac nerves affect myocardial stunning through reactive oxygen and nitric oxide mechanisms.
2003-10-31
Heterophase ligand exchange and metal transfer between monolayer protected clusters.
2003-09-24
Modifications of myosin-regulatory light chain correlate with function of stunned myocardium.
2003-07
Cystine crystal volume determination: a useful tool in the management of cystinuric patients.
2003-07
Adenosine and opioid receptor-mediated cardioprotection in the rat: evidence for cross-talk between receptors.
2003-07
Oxygen dependency of the adrenergic Na/H exchange in rainbow trout erythrocytes is diminished by a hydroxyl radical scavenger.
2003-06
[An observation of the effects of Tiopronin on the oxygen free radicals in severely scalded rats receiving delayed fluid resuscitation].
2003-04
Mitochondrial adenosine triphosphate-regulated potassium channel opening acts as a trigger for isoflurane-induced preconditioning by generating reactive oxygen species.
2003-04
Cardiac ischemia causes inhibition of the Na/K ATPase by a labile cytosolic compound whose production is linked to oxidant stress.
2003-03-15
[Cystinuria - Cystine Stones: Recommendations for Diagnosis, Therapy and Follow-up].
2003-03
[Long-term study of tiopronin in patients with cystinuria].
2003-02
Involvement of reactive oxygen species in cardiac preconditioning in rats.
2003-02
Effects of N-(2-mercaptopropionyl)-glycine on mitochondrial function in ischemic-reperfused heart.
2003-02
The cardioprotective and mitochondrial depolarising properties of exogenous nitric oxide in mouse heart.
2003-02
Paradoxical effects of thiol reagents on Jurkat cells and a new thiol-sensitive mutant form of human mitochondrial superoxide dismutase.
2003-01-01
Possible underlying mechanism for hydrogen peroxide-induced electromechanical suppression in human atrial myocardium.
2003-01
Effects of sulfhydryl compounds on pancreatic cytoprotection in acute necrotic pancreatitis.
2003-01
Meprin proteolytic complexes at the cell surface and in extracellular spaces.
2003
Enhancement of antiradiation potential of some aminothiols by beta-carotene.
2003
Patents

Patents

US7662987
3

Sample Use Guides

In Vivo Use Guide
Curator's Comment: Urinary cystine should be measured at 1 month after THIOLA treatment, and every 3 months thereafter. THIOLA dosage should be readjusted depending on the urinary cystine value.
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
Created
by admin
on Mon Mar 31 19:14:50 GMT 2025
Edited
by admin
on Mon Mar 31 19:14:50 GMT 2025
Record UNII
306C40V64U
Record Status Validated (UNII)
Record Version
  • Download
Name Type Language
ARGININE TIOPRONINE
WHO-DD  
Preferred Name English
TIOPRONIN ARGININE
Common Name English
L-ARGININE, COMPD. WITH N-(2-MERCAPTO-1-OXOPROPYL)GLYCINE (1:1)
Systematic Name English
GLYCINE, N-(2-MERCAPTO-1-OXOPROPYL)-, COMPD. WITH L-ARGININE (1:1)
Systematic Name English
Arginine tiopronine [WHO-DD]
Common Name English
ARGININE TIOPRONIN
Common Name English
Code System Code Type Description
FDA UNII
306C40V64U
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
PUBCHEM
56843946
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
EVMPD
SUB00582MIG
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
EPA CompTox
DTXSID90988585
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
SMS_ID
100000085321
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
CAS
68883-14-7
Created by admin on Mon Mar 31 19:14:50 GMT 2025 , Edited by admin on Mon Mar 31 19:14:50 GMT 2025
PRIMARY
Related Record Type Details
Structure of Tiopronin
PARENT -> SALT/SOLVATE
Related Record Type Details
Structure of Tiopronin
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