| Drug Name |
Tiopronin |
| Drug ID |
BADD_D02223 |
| 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 may also be used to bind metal nanoparticles in Wilson's disease, which is an overload of copper in the body. It has been investigated for use in the treatment of arthritis and as a neuroprotective agent in aneurysmal subarachnoid hemorrhage. |
| Indications and Usage |
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 increased fluid intake, decreased sodium and protein intake, and urine alkalinization. |
| Marketing Status |
approved; investigational |
| ATC Code |
G04BX16 |
| DrugBank ID |
DB06823
|
| KEGG ID |
D01430
|
| MeSH ID |
D008625
|
| PubChem ID |
5483
|
| TTD Drug ID |
D0A8CJ
|
| NDC Product Code |
0093-7909; 69766-033; 43235-0008; 43744-574; 0178-0900; 69575-4037; 58159-057; 0178-0901; 0178-0902; 75877-0004 |
| UNII |
C5W04GO61S
|
| Synonyms |
Tiopronin | 2-Thiolpropionamidoacetic Acid | 2 Thiolpropionamidoacetic Acid | Acid, 2-Thiolpropionamidoacetic | alpha-Mercaptopropionylglycine | alpha Mercaptopropionylglycine | Tiopronine | 2-Thiol-propionamido-acetic Acid | 2 Thiol propionamido acetic Acid | Acid, 2-Thiol-propionamido-acetic | Thiopronine | 2-Mercaptopropionylglycine | 2 Mercaptopropionylglycine | Mercaptopropionylglycine | Acadione | Thiola | Captimer | Meprin |
