ArticleJournal of the American Society of Nephrology : JASN2025
Misprocessing of α -Galactosidase A, Endoplasmic Reticulum Stress, and the Unfolded Protein Response.
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Article in Journal of the American Society of Nephrology : JASN, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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16citing papers in PubMed
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Who cites it
16 citing papers in PubMed.
- Clinical manifestations, diagnosis, and management of renal involvement in Fabry disease.Renal failure · 2026Review
- The Sphingolipid Balance and Endothelial Dysfunction in Lysosomal Storage Diseases: Shared Mechanisms in Gaucher, Niemann-Pick and Fabry Disease.International journal of molecular sciences · 2026Review
- Tri-Parametric Assessment of α-Galactosidase A Activity, lysoGb3 and X-Inactivation Aids Genotype-Phenotype Categorization of Fabry Disease Female Patients.Journal of inherited metabolic disease · 2026Article
- Modeling Fabry nephropathy: insights from experimental systems towards precision nephrology.Disease models & mechanisms · 2026Review
- IRE1-XBP1driven induction of TMED9 stabilizes ATF6 during ER stress to promote cell survival.Cellular & molecular biology letters · 2026Article
- NUFIP1-engineered exosomes modulate propofol-induced neurotoxicity in neonatal rats via the ERS apoptotic pathway.Apoptosis : an international journal on programmed cell death · 2026Article
- Pathogenic mechanisms in Fabry disease.Frontiers in medicine · 2026Review
- Sphingolipid metabolic enzyme GLA expression in gliomas: prognostic implications and therapeutic potential.Frontiers in oncology · 2026Article
- Cardiac manifestations of Fabry disease.NPJ cardiovascular health · 2025Review
- Two decades of experience of the Fabry Outcome Survey provides further confirmation of the long-term effectiveness of agalsidase alfa enzyme replacement therapy.Molecular genetics and metabolism reports · 2025Article
- Czech nationwide screening for Fabry disease in patients on maintenance dialysis: a call for evaluation of population-enrichedClinical kidney journal · 2025Article
- Impact of ER stress and the unfolded protein response on Fabry disease.EBioMedicine · 2025Review
- Pathogenesis of Organ Damage in Fabry Disease: The AGALopathy Pathogenetic Pathway.Journal of the American Society of Nephrology : JASN · 2025Article
- Opening New Routes for Kidney Therapy.Journal of the American Society of Nephrology : JASN · 2025Article
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Corrections and comments
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Authors and funding
33 authors.
Martina ŽivnáResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0003-1968-824
Gabriela DostálováSecond Department of Internal Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.ORCID 0000-0003-4809-1274
Veronika BarešováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0001-8989-6090
Dita MušálkováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Klára SvojšováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Doria MeiselesThe Mina and Everard Goodman Faculty of Life Sciences, Bar-Ilan University, Ramat-Gan, Israel.ORCID 0009-0002-3096-8277
Sara KinstlingerThe Mina and Everard Goodman Faculty of Life Sciences, Bar-Ilan University, Ramat-Gan, Israel.ORCID 0000-0001-5453-5957
Ladislav KuchařResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0003-0721-6624
Befekadu AsfawResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-3270-2192
Helena PoupětováDepartment of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic.ORCID 0000-0002-8230-757
Hana VláškováDiagnostic Laboratory, Department of Pediatrics and Inherited Metabolic Disorders, General University Hospital, Prague, Czech Republic.ORCID 0000-0003-4197-3100
Tereza KmochováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0009-0001-6677-7029
Petr VyleťalResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-9357-1237
Hana HartmannováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0001-7787-832
Kateřina HodaňováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Viktor StráneckýResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-2599-6479
Lenka Steiner-MrázováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0003-4347-7768
Aleš HnízdaResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Jan ŽivnýResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-1811-8977
Martin RadinaResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0009-0001-0027-8665
Miroslav VotrubaResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Jana SovováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Helena TrešlováResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Larisa StolnajaDiagnostic Laboratory, Department of Pediatrics and Inherited Metabolic Disorders, General University Hospital, Prague, Czech Republic.
Petra RekováDepartment of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic.ORCID 0000-0001-9881-4982
Lenka RoblováSecond Department of Internal Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.ORCID 0000-0003-0184-7003
Eva HonsováAeskuLab Pathology, Prague, Czech Republic.
Ivan RychlíkDepartment of Medicine, Third Faculty of Medicine, Charles University in Prague and Faculty Hospital Kralovske Vinohrady, Prague, Czech Republic.ORCID 0000-0002-4245-5949
Moran Dvela-LevittThe Mina and Everard Goodman Faculty of Life Sciences, Bar-Ilan University, Ramat-Gan, Israel.ORCID 0000-0001-5597-6455
Anthony J BleyerResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-2804-5273
Aleš LinhartSecond Department of Internal Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-3372-7850
Jakub SikoraResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Stanislav KmochResearch Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.ORCID 0000-0002-6239-707
Funding
Charles University in Prague UNCE 24/MED/022European Union - Next Generation EU from the Ministry of Education, Youth and Sports of the Czech Republicy LX22NPO5104European Union's Horizon 2020 Research and Innovation Programme under the Marie Skłodowska-Curie Grant Agreement 101003406 (HIPPOSTRUCT)Ministerstvo Zdravotnictví Ceské Republiky NU21-08-00324Ministry of Education, Youth and Sports of the Czech Republic LM2023067Ministry of Health of the Czech Republic NU21-07-00033
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