Tiopronin
title: "Tiopronin" type: doc version: 1 created: 2026-02-28 author: "Wikipedia contributors" status: active scope: public tags: ["alpha-amino-acids", "amino-acid-derivatives", "orphan-drugs", "thiols", "multiple-chemboxes"] topic_path: "general/alpha-amino-acids" source: "https://en.wikipedia.org/wiki/Tiopronin" license: "CC BY-SA 4.0" wikipedia_page_id: 0 wikipedia_revision_id: 0
::data[format=table title="Infobox drug"]
| Field | Value |
|---|---|
| type | |
| tradename | Thiola |
| licence_EU | |
| DailyMedID | Tiopronin |
| licence_US | |
| pregnancy_AU | |
| ATC_prefix | |
| legal_AU | |
| legal_BR | |
| legal_CA | |
| legal_DE | |
| legal_NZ | |
| legal_UK | |
| legal_US | |
| legal_UN | |
| legal_status | |
| DrugBank | DB06823 |
| ChEBI | 32229 |
| C | |
| K | |
| :: |
| Watchedfields = changed | verifiedrevid = 470610633 | ImageFile = Tiopronin.svg | ImageClass = skin-invert-image | ImageFile_Ref = | ImageName = Skeletal formula of tiopronin | IUPACName = N-(2-Sulfanylpropanoyl)glycine | SystematicName = (2-Sulfanylpropanamido)acetic acid | OtherNames = 2-mercaptopropionylglycine Acadione |Section1={{Chembox Identifiers | CASNo = 1953-02-2 | CASNo_Ref = | CASNo1 = 29335-92-0 | CASNo1_Ref = | CASNo1_Comment = R | PubChem = 5483 | PubChem1 = 208825 | PubChem1_Comment = R | PubChem2 = 736152 | PubChem2_Comment = S | ChemSpiderID = 5283 | ChemSpiderID_Ref = | ChemSpiderID1 = 180938 | ChemSpiderID1_Ref = | ChemSpiderID1_Comment = R | ChemSpiderID2 = 643292 | ChemSpiderID2_Ref = | ChemSpiderID2_Comment = S | UNII = C5W04GO61S | UNII_Ref = | UNII1_Ref = | UNII1 = X294K8K2PF | UNII1_Comment = R | EINECS = 217-778-4 | KEGG = D01430 | KEGG_Ref = | MeSHName = Tiopronin | ChEMBL = 1314 | ChEMBL_Ref = | RTECS = MC0596500 | Beilstein = 1859822 | SMILES = CC(S)C(=O)NCC(O)=O | StdInChI = 1S/C5H9NO3S/c1-3(10)5(9)6-2-4(7)8/h3,10H,2H2,1H3,(H,6,9)(H,7,8) | StdInChI_Ref = | StdInChIKey = YTGJWQPHMWSCST-UHFFFAOYSA-N | StdInChIKey_Ref = |Section2={{Chembox Properties | C=5 | H=9 | N=1 | S=1 | O=3 | Appearance = White, opaque crystals | MeltingPtC = 93 to 98 | LogP = −0.674 | pKa = 3.356 | pKb = 10.641 | Section6 = {{Chembox Pharmacology | Pharmacology_ref = | ATCCode_prefix = G04 | ATCCode_suffix = BX16 | ATC_Supplemental = | ATCvet = | Licence_EU = | INN = | INN_EMA = | Licence_US = | Legal_status = | Legal_AU = | Legal_AU_comment = | Legal_CA = | Legal_CA_comment = | Legal_NZ = | Legal_NZ_comment = | Legal_UK = | Legal_UK_comment = | Legal_US = Rx-only | Legal_US_comment = | Legal_EU = | Legal_EU_comment = | Legal_UN = | Legal_UN_comment = | Pregnancy_category = | Pregnancy_AU = | Pregnancy_AU_comment = | Dependence_liability = | AdminRoutes = By mouth | Bioavail = | ProteinBound = | Metabolism = | Metabolites = | OnsetOfAction = | HalfLife = | DurationOfAction = | Excretion = |Section7={{Chembox Hazards | GHSPictograms = | GHSSignalWord = WARNING | HPhrases = | LD50 = 1,300 mg kg−1 (oral, rat) |Section8={{Chembox Related | OtherFunction_label = alkanoic acids | OtherFunction = | OtherCompounds =
| drug_name = | INN = | type = | image = | width = | alt = | caption =
| pronounce = | tradename = Thiola | Drugs.com = | MedlinePlus = | licence_EU = | DailyMedID = Tiopronin | licence_US = | pregnancy_AU = | pregnancy_AU_comment = | pregnancy_category= | dependency_liability = | class = | ATCvet = | ATC_prefix = | ATC_suffix = | ATC_supplemental =
| legal_AU = | legal_AU_comment = | legal_BR = | legal_BR_comment = | legal_CA = | legal_CA_comment = | legal_DE = | legal_DE_comment = | legal_NZ = | legal_NZ_comment = | legal_UK = | legal_UK_comment = | legal_US = | legal_US_comment = | legal_EU = | legal_EU_comment = | legal_UN = | legal_UN_comment = | legal_status =
| bioavailability = | protein_bound = | metabolism = | metabolites = | onset = | elimination_half-life = | duration_of_action = | excretion =
| CAS_number_Ref = | CAS_number = | CAS_supplemental = | PubChem = | IUPHAR_ligand = | DrugBank_Ref = | DrugBank = DB06823 | ChemSpiderID_Ref = | ChemSpiderID = | UNII_Ref = | UNII = | KEGG_Ref = | KEGG = | KEGG2_Ref = | KEGG2 = | ChEBI_Ref = | ChEBI = 32229 | ChEMBL_Ref = | ChEMBL = | NIAID_ChemDB = | PDB_ligand = | synonyms =
| IUPAC_name = | chemical_formula_ref = | chemical_formula = | C= | H= | Ag= | Al= | As= | Au= | B= | Bi= | Br= | Ca= | Cl= | Co= | F= | Fe= | Gd= | I= | K= | Li= | Mg= | Mn= | N= | Na= | O= | P= | Pt= | S= | Sb= | Se= | Sr= | Tc= | Zn= | charge= | molecular_weight = | molecular_weight_comment = | SMILES = | StdInChI = | StdInChI_comment = | StdInChIKey = | density = | density_notes = | melting_point = | melting_high = | melting_notes = | boiling_point = | boiling_notes = | solubility = | sol_units = | specific_rotation =
Tiopronin, sold under the brand name Thiola, is a medication used to control the rate of cystine precipitation and excretion in the disease cystinuria.
It is available as a generic medication.
Medical uses
Tiopronin is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in people 20 kg and greater with severe homozygous cystinuria, who are not responsive to these measures alone.
Side effects
Tiopronin may present a variety of side effects, which are broadly similar to those of D-penicillamine and other compounds containing active sulfhydryl groups. Its pharmacokinetics have been studied.
Pharmacology
Mechanism of action
Tiopronin works by reacting with urinary cysteine to form a more soluble, disulfide linked, tiopronin-cysteine complex.
Society and culture
In the U.S., the drug was marketed by Mission Pharmacal at $1.50 per pill, but in 2014 the rights were bought by Retrophin, owned by Martin Shkreli, and the price increased to $30 per pill for a 100 mg capsule.
In 2016 Imprimis Pharmaceuticals introduced a lower cost version marketed as a compounded drug.
Research
It may also be used for Wilson's disease (an overload of copper in the body), and has also been investigated for the treatment of arthritis, though tiopronin is not an anti-inflammatory.
Tiopronin is also sometimes used as a stabilizing agent for metal nanoparticles. The thiol group binds to the nanoparticles, preventing coagulation.
References
References
- "Thiola- tiopronin tablet, sugar coated".
- (15 March 2021). "Thiola EC- tiopronin tablet, delayed release".
- (1995). "Clinical course and cystine stone formation during tiopronin treatment". Urological Research.
- (October 1992). "The pathogenesis and treatment of kidney stones". The New England Journal of Medicine.
- (29 June 2023). "Competitive Generic Therapy Approvals".
- (30 May 2023). "First Generic Drug Approvals 2023".
- (March 1986). "Adverse effects profile of sulfhydryl compounds in man". The American Journal of Medicine.
- (August 1993). "Pharmacokinetics of oral tiopronin". European Journal of Clinical Pharmacology.
- (11 September 2014). "The Most Unconscionable Drug Price Hike I Have Yet Seen". In the Pipeline.
- (September 20, 2015). "Why would Martin Shkreli hike an old drug price by 5000%? Only a 'moron' would ask". FierceBiotech.
- (September 29, 2016). "Imprimis shuts down Texas plant, axes 8% of jobs". BioPharma Dive.
- (April 1989). "[Tolerability and therapeutic maintenance of tiopronin, new basic treatment of rheumatoid arthritis. Apropos of long-term follow-up of 268 cases]". Revue du Rhumatisme et des Maladies Osteo-Articulaires.
- (August 1982). "Controlled multicenter trial of tiopronin and d-penicillamine for rheumatoid arthritis". Arthritis and Rheumatism.
- (June 2007). "Toward greener nanosynthesis". Chemical Reviews.
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