{{chembox | Watchedfields = changed | verifiedrevid = 470610633 | ImageFile = Tiopronin.svg | ImageClass = skin-invert-image | ImageFile_Ref = {{chemboximage|correct|??}} | ImageName = Skeletal formula of tiopronin | IUPACName = ''N''-(2-Sulfanylpropanoyl)glycine | SystematicName = (2-Sulfanylpropanamido)acetic acid | OtherNames = 2-mercaptopropionylglycine<br />Acadione |Section1={{Chembox Identifiers | CASNo = 1953-02-2 | CASNo_Ref = {{cascite|correct|CAS}} | CASNo1 = 29335-92-0 | CASNo1_Ref = {{cascite|correct|CAS}} | CASNo1_Comment = <small>''R''</small> | PubChem = 5483 | PubChem1 = 208825 | PubChem1_Comment = <small>''R''</small> | PubChem2 = 736152 | PubChem2_Comment = <small>''S''</small> | ChemSpiderID = 5283 | ChemSpiderID_Ref = {{chemspidercite|correct|chemspider}} | ChemSpiderID1 = 180938 | ChemSpiderID1_Ref = {{chemspidercite|correct|chemspider}} | ChemSpiderID1_Comment = <small>''R''</small> | ChemSpiderID2 = 643292 | ChemSpiderID2_Ref = {{chemspidercite|correct|chemspider}} | ChemSpiderID2_Comment = <small>''S''</small> | UNII = C5W04GO61S | UNII_Ref = {{fdacite|correct|FDA}} | UNII1_Ref = {{fdacite|correct|FDA}} | UNII1 = X294K8K2PF | UNII1_Comment = <small>''R''</small> | EINECS = 217-778-4 | KEGG = D01430 | KEGG_Ref = {{keggcite|correct|kegg}} | MeSHName = Tiopronin | ChEMBL = 1314 | ChEMBL_Ref = {{ebicite|correct|EBI}} | 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 = {{stdinchicite|correct|chemspider}} | StdInChIKey = YTGJWQPHMWSCST-UHFFFAOYSA-N | StdInChIKey_Ref = {{stdinchicite|correct|chemspider}} }} |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|G04|BX16}} | 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 = <ref name="Thiola FDA label">{{cite web | title=Thiola- tiopronin tablet, sugar coated | website=DailyMed | url=https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=494a714e-923c-cd57-df6c-12886afb265a | access-date=18 June 2021}}</ref><ref name="Thiola EC FDA label">{{cite web | title=Thiola EC- tiopronin tablet, delayed release | website=DailyMed | date=15 March 2021 | url=https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=20298a52-a194-a161-8632-d84b6f26e23c | access-date=6 March 2023}}</ref> | 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 = {{gHS exclamation mark}} | GHSSignalWord = '''WARNING''' | HPhrases = {{h-phrases|302}} | LD50 = 1,300 mg kg<sup>−1</sup> <small>(oral, rat)</small> }} |Section8={{Chembox Related | OtherFunction_label = alkanoic acids | OtherFunction = {{unbulleted list|Acetylcysteine|Glycylglycine|Iminodiacetic acid|Nitrilotriacetic acid|''N''-Oxalylglycine|Bucillamine|Oxalyldiaminopropionic acid|''gamma''-Glutamylcysteine}} | OtherCompounds = {{unbulleted list|''N''-Acetylglycinamide}} }} }}
{{Infobox drug | drug_name = | INN = | type = <!-- empty --> | image = | width = | alt = | caption =
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'''Tiopronin''', sold under the brand name '''Thiola''', is a medication used to control the rate of cystine precipitation and excretion in the disease cystinuria.<ref>{{cite journal | vauthors = Lindell A, Denneberg T, Hellgren E, Jeppsson JO, Tiselius HG | title = Clinical course and cystine stone formation during tiopronin treatment | journal = Urological Research | volume = 23 | issue = 2 | pages = 111–7 | year = 1995 | pmid = 7676533 | doi = 10.1007/BF00307941 | s2cid = 34308815 }}</ref><ref>{{cite journal | vauthors = Coe FL, Parks JH, Asplin JR | title = The pathogenesis and treatment of kidney stones | journal = The New England Journal of Medicine | volume = 327 | issue = 16 | pages = 1141–52 | date = October 1992 | pmid = 1528210 | doi = 10.1056/NEJM199210153271607 }}</ref>
It is available as a generic medication.<ref>{{cite web | title=Competitive Generic Therapy Approvals | website=U.S. Food and Drug Administration (FDA) | date=29 June 2023 | url=https://www.fda.gov/drugs/generic-drugs/competitive-generic-therapy-approvals | access-date=29 June 2023 | archive-date=29 June 2023 | archive-url=https://web.archive.org/web/20230629233651/https://www.fda.gov/drugs/generic-drugs/competitive-generic-therapy-approvals | url-status=dead }}</ref><ref>{{cite web | title=First Generic Drug Approvals 2023 | website=U.S. Food and Drug Administration (FDA) | date=30 May 2023 | url=https://www.fda.gov/drugs/drug-and-biologic-approval-and-ind-activity-reports/first-generic-drug-approvals | archive-url=https://web.archive.org/web/20230630003621/https://www.fda.gov/drugs/drug-and-biologic-approval-and-ind-activity-reports/first-generic-drug-approvals | archive-date=30 June 2023 | url-status=live | access-date=30 June 2023}}</ref>
==Medical uses== Tiopronin is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in people {{convert|20|kg|lb}} and greater with severe homozygous cystinuria, who are not responsive to these measures alone.<ref name="Thiola FDA label" /><ref name="Thiola EC FDA label" />
==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.<ref>{{cite journal | vauthors = Jaffe IA | title = Adverse effects profile of sulfhydryl compounds in man | journal = The American Journal of Medicine | volume = 80 | issue = 3 | pages = 471–6 | date = March 1986 | pmid = 2937293 | doi = 10.1016/0002-9343(86)90722-9 }}</ref> Its pharmacokinetics have been studied.<ref name="Carlsson" />
== Pharmacology == === Mechanism of action === Tiopronin works by reacting with urinary cysteine to form a more soluble, disulfide linked, tiopronin-cysteine complex.<ref name="Carlsson">{{cite journal | vauthors = Carlsson MS, Denneberg T, Emanuelsson BM, Kågedal B, Lindgren S | title = Pharmacokinetics of oral tiopronin | journal = European Journal of Clinical Pharmacology | volume = 45 | issue = 1 | pages = 79–84 | date = August 1993 | pmid = 8405034 | doi = 10.1007/BF00315354 | s2cid = 8879752 }}</ref>
==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.<ref>{{cite news| vauthors = Lowe D |title=The Most Unconscionable Drug Price Hike I Have Yet Seen|url=https://www.science.org/content/blog-post/most-unconscionable-drug-price-hike-i-have-yet-seen|work=In the Pipeline|date=11 September 2014}}</ref><ref>{{cite web|url=http://www.fiercebiotech.com/story/why-would-martin-shkreli-hike-old-drug-price-5000-only-moron-would-ask/2015-09-20|title=Why would Martin Shkreli hike an old drug price by 5000%? Only a 'moron' would ask|work=FierceBiotech | vauthors = Carroll J |date=September 20, 2015}}</ref>
In 2016 Imprimis Pharmaceuticals introduced a lower cost version marketed as a compounded drug.<ref>{{cite news| vauthors = Elvidge S |title=Imprimis shuts down Texas plant, axes 8% of jobs|url=https://www.biopharmadive.com/news/imprimis-shuts-down-texas-plant-axes-8-of-jobs/427296/|work=BioPharma Dive|date=September 29, 2016}}</ref>
== 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,<ref>{{cite journal | vauthors = Delecoeuillerie G | title = [Tolerability and therapeutic maintenance of tiopronin, new basic treatment of rheumatoid arthritis. Apropos of long-term follow-up of 268 cases] | journal = Revue du Rhumatisme et des Maladies Osteo-Articulaires | volume = 56 | issue = 5 Pt 2 | pages = 38–42 | date = April 1989 | pmid = 2740804 }}</ref><ref>{{cite journal | vauthors = Pasero G, Pellegrini P, Ambanelli U, Ciompi ML, Colamussi V, Ferraccioli G, Barbieri P, Mazzoni MR, Menegale G, Trippi D | display-authors = 6 | title = Controlled multicenter trial of tiopronin and d-penicillamine for rheumatoid arthritis | journal = Arthritis and Rheumatism | volume = 25 | issue = 8 | pages = 923–9 | date = August 1982 | pmid = 7115451 | doi = 10.1002/art.1780250803 | doi-access = }}</ref> though tiopronin is not an anti-inflammatory.{{citation needed|date=June 2021}}
Tiopronin is also sometimes used as a stabilizing agent for metal nanoparticles. The thiol group binds to the nanoparticles, preventing coagulation.<ref>{{cite journal | vauthors = Dahl JA, Maddux BL, Hutchison JE | title = Toward greener nanosynthesis | journal = Chemical Reviews | volume = 107 | issue = 6 | pages = 2228–69 | date = June 2007 | pmid = 17564480 | doi = 10.1021/cr050943k | name-list-style = amp | citeseerx = 10.1.1.454.2724 }}</ref>
== References == {{reflist}}
{{Urologicals, including antispasmodics}} {{Portal bar | Medicine}}
Category:Alpha-Amino acids Category:Amino acid derivatives Category:Orphan drugs Category:Thiols X