ArticleJournal of the American Society of Nephrology : JASN2014
Cysteamine modulates oxidative stress and blocks myofibroblast activity in CKD.
Article in Journal of the American Society of Nephrology : JASN, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 1 of them a synthesis that pooled it.
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Who cites it
37 citing papers in PubMed, 1 synthesis or guideline pooled it, 75 citations in OpenAlex.
- Revealing VNN1: An Emerging and Promising Target for Inflammation and Redox Balance.Immunity, inflammation and disease · 2025Pooled it
- The response to kidney injury is epigenetically regulated through the activation of bivalent genes.American journal of physiology. Renal physiology · 2026Article
- A comprehensive approach to elucidating the pathophysiology of kidney fibrosis based on extracellular vesicle proteomics.Frontiers in physiology · 2026Article
- Senescent macrophages in tumor: phenotypes, roles, and interventions.Cell death & disease · 2025Review
- From Molecular Understanding and Pathophysiology to Disease Management; A Practical Approach and Guidance to the Management of the Ocular Manifestations of Cystinosis.International journal of molecular sciences · 2025Review
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- Article
- Mitigative role of cysteamine against unilateral renal reperfusion injury in Wistar rats.Frontiers in pharmacology · 2024Article
- A Review on the Antimutagenic and Anticancer Effects of Cysteamine.Advances in pharmacological and pharmaceutical sciences · 2023Review
- Exploring antiviral and anti-inflammatory effects of thiol drugs in COVID-19.American journal of physiology. Lung cellular and molecular physiology · 2022Article
- Inhibiting Transglutaminase 2 Mediates Kidney Fibrosis via Anti-Apoptosis.Biomedicines · 2022Article
- Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies.Cells · 2022Review
- Cysteamine affects skeletal development and impairs motor behavior in zebrafish.Frontiers in pharmacology · 2022Article
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- Spiny mice activate unique transcriptional programs after severe kidney injury regenerating organ function without fibrosis.iScience · 2021Article
- Maternal supply of cysteamine alleviates oxidative stress and enhances angiogenesis in porcine placenta.Journal of animal science and biotechnology · 2021Article
- Effects of Coated Cysteamine on Oxidative Stress and Inflammation in Weaned Pigs.Animals : an open access journal from MDPI · 2021Article
- Recombinant Long-Acting Thioredoxin Ameliorates AKI to CKD Transition via Modulating Renal Oxidative Stress and Inflammation.International journal of molecular sciences · 2021Article
- Fenofibrate Protects Cardiomyocytes from Hypoxia/Reperfusion- and High Glucose-Induced Detrimental Effects.PPAR research · 2021Article
- GSTA3 regulates TGF-The Journal of international medical research · 2019Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
Therapy to slow the relentless expansion of interstitial extracellular matrix that leads to renal functional decline in patients with CKD is currently lacking. Because chronic kidney injury increases tissue oxidative stress, we evaluated the antifibrotic efficacy of cysteamine bitartrate, an antioxidant therapy for patients with nephropathic cystinosis, in a mouse model of unilateral ureteral obstruction. Fresh cysteamine (600 mg/kg) was added to drinking water daily beginning on the day of surgery, and outcomes were assessed on days 7, 14, and 21 after surgery. Plasma cysteamine levels showed diurnal variation, with peak levels similar to those observed in patients with cystinosis. In cysteamine-treated mice, fibrosis severity decreased significantly at 14 and 21 days after unilateral ureteral obstruction, and renal oxidized protein levels decreased at each time point, suggesting reduced oxidative stress. Consistent with these results, treatment of cultured macrophages with cysteamine reduced cellular generation of reactive oxygen species. Furthermore, treatment with cysteamine reduced α-smooth muscle actin-positive interstitial myofibroblast proliferation and mRNA levels of extracellular matrix proteins in mice and attenuated myofibroblast differentiation and proliferation in vitro, but did not augment TGF-β signaling. In a study of renal ischemia reperfusion, cysteamine therapy initiated 10 days after injury and continued for 14 days decreased renal fibrosis by 40%. Taken together, these data suggest previously unrecognized antifibrotic actions of cysteamine via TGF-β-independent mechanisms that include oxidative stress reduction and attenuation of the myofibroblast response to kidney injury and support further investigation into the potential benefit of cysteamine therapy in the treatment of CKD.
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