ArticleKidney international2019
Interaction between galectin-3 and cystinosin uncovers a pathogenic role of inflammation in kidney involvement of cystinosis.
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Article in Kidney international, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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23citing papers in PubMed
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23 citing papers in PubMed, 40 citations in OpenAlex.
- Cystinosin regulates NaEMBO reports · 2026Article
- Cystinosin/Ers1 functions in redox homeostasis in the early secretory pathway.bioRxiv : the preprint server for biology · 2026Article
- Cystinosis and Cellular Energy Failure: Mitochondria at the Crossroads.International journal of molecular sciences · 2026Review
- Serum N-glycosylation is altered in Nephropathic Cystinosis.Glycobiology · 2025Article
- 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
- TRPML-1 Dysfunction and Renal Tubulopathy in Mucolipidosis Type IV.Journal of the American Society of Nephrology : JASN · 2025Article
- Cystinosin is involved in NabioRxiv : the preprint server for biology · 2025Article
- Novel insights into the association between organ damage and inflammatory response in preoperative abdominal aortic aneurysms.Frontiers in cardiovascular medicine · 2025Article
- Ketogenic Diet and Progression of Kidney Disease in Animal Models of Nephropathic Cystinosis.Journal of the American Society of Nephrology : JASN · 2024Article
- Article
- Catalpol Prevents Glomerular Angiogenesis Induced by Advanced Glycation End Products via Inhibiting Galectin-3.Current medical science · 2023Article
- Article
- Review
- Activation of the NLRP3 inflammasome by RAC1 mediates a new mechanism in diabetic nephropathy.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2022Article
- Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies.Cells · 2022Review
- Drug Repurposing in Rare Diseases: An Integrative Study of Drug Screening and Transcriptomic Analysis in Nephropathic Cystinosis.International journal of molecular sciences · 2021Article
- Galectin-3 and Risk of Late Graft Failure in Kidney Transplant Recipients: A 10-year Prospective Cohort Study.Transplantation · 2021Observational
- Article
- CRISPR-Cas9 Gene Editing of Hematopoietic Stem Cells from Patients with Friedreich's Ataxia.Molecular therapy. Methods & clinical development · 2020Article
- Microvesicle delivery of a lysosomal transport protein to ex vivo rabbit cornea.Molecular genetics and metabolism reports · 2020Article
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Authors and funding
17 authors at 7 institutions in 2 countries.
Tatiana LobryDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA; INSERM, U1078, Équipe 'Transfert de gènes et thérapie génique', Faculté de Médecine, Brest, France, and CHRU de Brest, Service de Génétique Moléculaire et d'histocompatibilité, Brest, France.
Roy MillerDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA.
Nathalie NevoINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France.
Celine J RoccaDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA.
Jinzhong ZhangDepartment of Molecular Medicine, The Scripps Research Institute, La Jolla, California, USA.
Sergio D CatzDepartment of Molecular Medicine, The Scripps Research Institute, La Jolla, California, USA.
Fiona MooreDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA.
Lucie ThomasINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France.
Daniel PoulyINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France.
Anne BailleuxINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France.
Ida Chiara GuerreraProteomics Platform 3P5-Necker, Université Paris Descartes-Structure Fédérative de Recherche Necker, INSERM US24/CNRS UMS3633, Paris, France.
Marie-Claire GublerINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France.
Wai W CheungDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA.
Robert H MakDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA.
Tristan MontierINSERM, U1078, Équipe 'Transfert de gènes et thérapie génique', Faculté de Médecine, Brest, France, and CHRU de Brest, Service de Génétique Moléculaire et d'histocompatibilité, Brest, France.
Corinne AntignacINSERM, U1163, Imagine Institute, Laboratory of Hereditary Kidney Diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Paris, France; Department of Genetics, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Stephanie CherquiDepartment of Pediatrics, Division of Genetics, University of California, San Diego, La Jolla, California, USA. Electronic address: scherqui@ucsd.edu.
University of California, San Diego · USInstitut des Maladies Génétiques Imagine · FRScripps Research Institute · USSorbonne Paris Cité · FRGénétique, Génomique Fonctionnelle et Biotechnologies · FRStructure fédérative de recherche NeckerUniversité Paris Cité · FR
Funding
U of Calif, San Diego Neuroscience Microscopy ImagingP30NS047101 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI GLEESON, JOSEPH G, ZHENG, BINHAI · 2003 to 2022
$9.0MVesicular trafficking mechanisms regulating granulocyte functionR01HL088256 · NHLBI · SCRIPPS RESEARCH INSTITUTE, THE · PI Sergio Daniel Catz · 2009 to 2026
$9.0MMolecular and Cellular Mechanisms of the Lysosomal Storage Disease CystinosisR01DK110162 · NIDDK · SCRIPPS RESEARCH INSTITUTE, THE · PI CATZ, SERGIO DANIEL, CHERQUI, STEPHANIE · 2017 to 2025
$4.7MLentiviral-transduced hematopoictic stem cell transplantation for cystinosisR01DK090058 · NIDDK · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI CHERQUI, STEPHANIE · 2011 to 2019
$2.7MChaperone-mediated autophagy in corneal cystinosisR21EY028642 · NEI · SCRIPPS RESEARCH INSTITUTE, THE · PI CATZ, SERGIO DANIEL, CHERQUI, STEPHANIE · 2018 to 2019
$541kNEI NIH HHS R21 EY028642NHLBI NIH HHS R01 HL088256NIDDK NIH HHS R01 DK090058NIDDK NIH HHS R01 DK110162NINDS NIH HHS P30 NS047101
Abstract
Inflammation is involved in the pathogenesis of many disorders. However, the underlying mechanisms are often unknown. Here, we test whether cystinosin, the protein involved in cystinosis, is a critical regulator of galectin-3, a member of the β-galactosidase binding protein family, during inflammation. Cystinosis is a lysosomal storage disorder and, despite ubiquitous expression of cystinosin, the kidney is the primary organ impacted by the disease. Cystinosin was found to enhance lysosomal localization and degradation of galectin-3. In Ctns
Indexed as
Amino Acid Transport Systems, NeutralAnimalsChemokine CCL2CystineCystinosisDisease Models, AnimalDisease ProgressionFanconi SyndromeFemaleGalectin 3HumansInflammationKidney Tubules, ProximalLysosomesMacrophagesMaleAmino Acid Transport Systems, NeutralCcl2 protein, mouseChemokine CCL2Cystinecystinosin protein, mouseGalectin 3Lgals3 protein, mousechronic kidney diseasecystinosisgalectin-3inflammationmonocyte chemoattractant protein–1
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