ArticleUrology2014
Novel cystine ester mimics for the treatment of cystinuria-induced urolithiasis in a knockout mouse model.
Article in Urology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
12 citing papers in PubMed, 24 citations in OpenAlex.
- 8-l-Cystinyl Bis(1,8-diazaspiro[4.5]decane) as an Orally Bioavailable l-Cystine Crystallization Inhibitor for Cystinuria.ACS medicinal chemistry letters · 2024Article
- A Summary of Current Guidelines and Future Directions for Medical Management and Monitoring of Patients with Cystinuria.Healthcare (Basel, Switzerland) · 2023Article
- Disrupting Crystal Growth through Molecular Recognition: Designer Therapies for Kidney Stone Prevention.Accounts of chemical research · 2022Article
- Update on cystine stones: current and future concepts in treatment.Intractable & rare diseases research · 2020Review
- Cystinuria: genetic aspects, mouse models, and a new approach to therapy.Urolithiasis · 2019Review
- Nephrolithiasis and Nephrocalcinosis in Childhood-Risk Factor-Related Current and Future Treatment Options.Frontiers in pediatrics · 2018Review
- Role of Molecular Recognition in l-Cystine Crystal Growth Inhibition.Crystal growth & design · 2017Article
- α-Lipoic acid treatment prevents cystine urolithiasis in a mouse model of cystinuria.Nature medicine · 2017Article
- l-Cystine Diamides as l-Cystine Crystallization Inhibitors for Cystinuria.Journal of medicinal chemistry · 2016Article
- Article
- Cystine growth inhibition through molecular mimicry: a new paradigm for the prevention of crystal diseases.Current rheumatology reports · 2015Review
- Cystine nephrolithiasis.Translational andrology and urology · 2014Article
Corrections and comments
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Authors and funding
15 authors at 4 institutions in 2 countries.
Funding
Abstract
objectiveTo assess the effectiveness of l-cystine dimethyl ester (CDME), an inhibitor of cystine crystal growth, for the treatment of cystine urolithiasis in an Slc3a1 knockout mouse model of cystinuria. MATERIALS AND
methodsCDME (200 μg per mouse) or water was delivered by gavage daily for 4 weeks. Higher doses by gavage or in the water supply were administered to assess organ toxicity. Urinary amino acids and cystine stones were analyzed to assess drug efficacy using several analytical methods.
resultsTreatment with CDME led to a significant decrease in stone size compared with that of the water group (P = .0002), but the number of stones was greater (P = .005). The change in stone size distribution between the 2 groups was evident by micro computed tomography. Overall, cystine excretion in urine was the same between the 2 groups (P = .23), indicating that CDME did not interfere with cystine metabolism. Scanning electron microscopy analysis of cystine stones from the CDME group demonstrated a change in crystal habit, with numerous small crystals. l-cysteine methyl ester was detected by ultra-performance liquid chromatography-mass spectrometer in stones from the CDME group only, indicating that a CDME metabolite was incorporated into the crystal structure. No pathologic changes were observed at the doses tested.
conclusionThese data demonstrate that CDME promotes formation of small stones but does not prevent stone formation, consistent with the hypothesis that CDME inhibits cystine crystal growth. Combined with the lack of observed adverse effects, our findings support the use of CDME as a viable treatment for cystine urolithiasis.
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Registered trials
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