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tiopronin (Thiola EC)

✓ Approved

Mission Pharmacal Company · Small Molecule · Small Molecule

What is tiopronin?

tiopronin is a small molecule developed by Mission Pharmacal Company. It is approved for therapeutic indications via oral (po).

Drug Profile

Brand NamesThiola EC
CompanyMission Pharmacal Company
Drug ClassSmall Molecule
RouteOral (PO)
StatusApproved

Therapeutic Indications

tiopronin is developed for 5 unique indications across 5 therapeutic areas.

Therapeutic AreaConditionPhase
Congenital, familial and genetic disordersCystinuriaBLA/NDA
Renal and urinary disordersCalculus urinaryPreclinical
Eye disordersCataractPreclinical
Injury, poisoning and procedural complicationsMetal poisoningPreclinical
Musculoskeletal and connective tissue disordersRheumatoid arthritisPreclinical

Related Research Articles

PubMedFrontiers in medicine2026-08-25

Case Report: Nephrotic syndrome with minimal change disease during anti-tuberculosis treatment and tiopronin use.

Liao Heng H, Yan Fei F, Liu Xingxia X

Hepatoprotective agents are often added during prolonged anti-tuberculosis (anti-TB) therapy, and medication-associated nephrotic syndrome may be under-recognized. A 31-year-old man with pulmonary tuberculosis received long-term anti-TB therapy (rifapentine, isoniazid, and ethambutol) and continuous tiopronin (0.2 g three times daily). He developed edema, foamy urine, and oliguria. Pre-admission tests showed proteinuria (3+), serum albumin 19.6 g/L, and serum creatinine 161 μmol/L. On admission, urine protein was 4+, serum albumin 20.6 g/L, serum creatinine 142.9 μmol/L, and estimated glomerular filtration rate (eGFR) 58.86 mL/min, with hyperlipidemia. Kidney biopsy confirmed minimal change disease (MCD). All anti-TB drugs and tiopronin were discontinued, and tacrolimus was initiated after biopsy. Anti-TB therapy was reintroduced as isoniazid monotherapy. Renal function improved rapidly (serum creatinine 55 μmol/L; eGFR 128.1 mL/min at discharge), and dipstick proteinuria became negative on short-term follow-up. The tacrolimus trough concentration was 3.6 ng/mL. The improvement after withdrawal of multiple suspected agents and continued recovery after reintroduction of isoniazid alone support a drug-associated etiology, but do not allow definitive attribution to a single causative drug. Routine renal monitoring should accompany hepatoprotective add-on therapy during anti-TB treatment.

PubMedMedicine2026-08-15

Tiopronin-induced reversible bilateral parotid gland enlargement in a patient with alcoholic cirrhosis: Case report.

Jiang Shunshun S, Chen Ge G, Kong Lingling L, Zhao Hong H

Drug-induced parotid gland enlargement is uncommon, and reports associated with tiopronin are extremely rare in both domestic and international literature. The mechanism remains unclear, and its rapid onset may easily lead to misdiagnosis as allergic reactions or other salivary gland diseases. Timely recognition and management are crucial to avoid unnecessary interventions. A 63-year-old man with alcoholic cirrhosis was admitted to our department for further management. Tiopronin was administered intravenously for hepatoprotective purposes. Approximately 20 minutes after the initiation of the infusion, the patient developed painless bilateral parotid gland swelling. Emergency drug withdrawal was carried out immediately, and an ultrasound examination demonstrated bilateral parotid gland enlargement without obvious inflammatory changes or ductal dilation, consistent with acute noninflammatory sialadenosis. The tiopronin infusion was discontinued promptly. No additional interventions were administered. The patient was monitored closely with serial physical examinations. During the evening ward round on the same day, a significant reduction in parotid swelling was observed. Two days later, bilateral parotid gland enlargement had nearly completely resolved without specific treatment. Tiopronin-induced acute parotid gland enlargement is extremely rare. This case highlights the importance of considering drug-induced sialadenosis as a differential diagnosis when acute parotid swelling occurs shortly after drug administration. Early identification and discontinuation of the suspected agent can lead to rapid resolution, avoiding unnecessary interventions. Clinicians should be aware of this potential reaction and strengthen pharmacovigilance during tiopronin use.

PubMedKidney & blood pressure research2026-06-30

Cystine-Binding Medication Is Poorly Accessible for the Treatment of Cystinuria.

Peng Xuemi X, Penninx Bart B, Marsman Roos F RF, Capolongo Giovanna G et al.

Cystinuria is a rare inherited disorder characterized by recurrent cystine stone formation. When lifestyle modification and urine alkalinization fail, cystine-binding medication such as tiopronin and D-penicillamine are indicated. Despite proven benefit, their accessibility across Europe appears limited. We hypothesized that access to this medication is restricted and varies substantially between European countries. We surveyed physicians affiliated with the European Reference Network for Rare Kidney Diseases (ERKNet) regarding access barriers and prescribing practices. The online questionnaire was completed by 40 clinicians from 15 countries. Eighty-five percent of respondents had prescribed cystine-binding medication in the past 2 years, with tiopronin being the preferred agent. However, 30% reported having no access to tiopronin at all, and 83% of recent prescribers experienced accessibility problems, including high costs and unfilled prescriptions. Substantial differences were observed both between and within countries. Prescription rates correlated with physicians' cystinuria caseload. Cystine-binding medication is poorly and inconsistently accessible across Europe. Coordinated efforts are urgently needed to stabilize supply and ensure equitable, affordable access.

PubMedTalanta2026-05-22

Development of two flow analysis methods for the determination of tiopronin in pharmaceuticals.

Kukoc Lea L, Biocic Maja M, Spassov Tony G TG, Kolev Spas D SD

A flow injection analysis (FIA) method and the first sequential injection analysis (SIA) method for the determination of tiopronin in pharmaceuticals have been developed. Both utilize the one-step colour reaction involving the reduction of the Cu(II)-bathocuproine complex by tiopronin to the orange-coloured Cu(I)-bathocuproine complex with maximum absorbance at 483 nm. The FIA and SIA methods exhibit linear calibration ranges of 4.0 × 10-7 - 4.0 × 10-5 and 4.0 × 10-7 - 6.0 × 10-5 mol L-1, repeatability (expressed as relative standard deviation) of 1.4 and 2.9 % and sampling rates of 180 and 36 h-1, respectively. Both methods are characterised by limits of detection and quantitation of 1.4 × 10-7 and 4.6 × 10-7 mol L-1, respectively. They have been successfully applied to the determination of tiopronin in a pharmaceutical. No statistically significant difference has been observed between the two methods, and between each one of them and the standard Pharmacopoeia method on one hand and the amount of tiopronin per tablet quoted by the tablet manufacturer. Good recovery results for both flow methods have been also obtained with spiked tablet samples. Based on the sampling rate and reagent consumption results, it has been concluded that the FIA method, which offers a higher sampling rate than existing FIA methods, is more suitable for the analysis of large numbers of samples while the SIA method minimises analysis costs and the generation of chemical waste.

PubMedOrphanet journal of rare diseases2026-04-23

A rapid HPLC method to simultaneously quantify therapeutic thiols and monitor their disulfide exchange reactions with cystine.

Hadjipour Azhidhack A, Gayatri Gayatri G, Rioux Patrice P, Kavanagh Oisín N ON

This study introduces a rapid and environmentally sustainable High Performance Liquid Chromatography method to simultaneously quantify free thiols and monitor their disulfide exchange kinetics with cystine. This method is based on the derivatisation of thiols with 5,5’-dithiobis(2-nitrobenzoic acid) (DTNB, also known as Ellman’s reagent). The method utilises a mobile phase consisting of 0.1% v/v Trifluoroacetic acid in water and methanol, with Ultraviolet detection at 248 nm. The method exhibited high sensitivity (Limit of Quantification 0.78 µM; Limit of Detection 0.39 µM) and selectivity for biologically relevant thiols (cysteamine, cysteine, tiopronin, glutathione, captopril and penicillamine) in an aqueous environment. Simultaneous quantification of these thiols was successfully achieved in phosphate-buffered saline as well as in biological samples such as human saliva and urine (from a vegetarian subject). Furthermore, it facilitated kinetic analysis of thiol-related reactions, with potential applications in both clinical and pharmacological research.

PubMedJournal of fluorescence2026-04-16

N-Doped CQDs as a Fluorescence Probe for Tiopronin Detection in Pharmaceutical Formulations via Static Quenching.

Liu Tianhui T, Yan Yiling Y, Chen Zhenni Z, Zhao Chengfei C

In recent years, fluorescent sensors based on carbon quantum dots (CQDs) have been widely reported for pharmaceutical detection owing to their numerous advantages. In this study, a Fe³⁺-mediated fluorescence “off–on” sensor based on nitrogen-doped carbon quantum dots (N-CQDs) was developed for the selective detection of tiopronin (TPN). Briefly, highly fluorescent N-CQDs were synthesized via a one-step hydrothermal method using citric acid monohydrate and diethylenetriamine. XPS, FT-IR, UV-Vis, fluorescence spectroscopy, and DLS confirmed that the N-CQDs exhibit uniform size, aqueous stability, excitation-independent fluorescence, and a high quantum yield of 62.99%. Fe³⁺ selectively bound to N-CQDs to form a complex, resulting in fluorescence quenching. Subsequently, upon the addition of TPN, the fluorescence of N-CQDs showed a proportional recovery. During this process, the competitive coordination between TPN and Fe³⁺ led to the release of Fe³⁺ from the N-CQDs, thereby restoring the quenched fluorescence. The limit of detection (LOD) of TPN was 80 μmol/L, with a linear range of 0.2–0.7 mmol/L and an R² value of 0.9993. The content of TPN in enteric-coated tablets was quantified using this method, yielding recovery rates ranging from 92.59% to 104.22%. This method is simple, rapid, and reliable, offering good sensitivity without the need for expensive reagents or complex equipment.

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