ArticleStem cell reports2018
Organoids from Nephrotic Disease-Derived iPSCs Identify Impaired NEPHRIN Localization and Slit Diaphragm Formation in Kidney Podocytes.
Article in Stem cell reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 89 papers.
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Who cites it
89 citing papers in PubMed, 142 citations in OpenAlex.
- Generation of the functionally enhanced proximal tubular cells from human iPSC-derived kidney organoids.Experimental & molecular medicine · 2026Article
- Kidney organoids as models for hereditary kidney diseases: Toward precision medicine (Review).Experimental and therapeutic medicine · 2026Review
- Coalescing nephron and ureteric bud progenitors potentiates nephrogenesis in recellularized kidney scaffolds.bioRxiv : the preprint server for biology · 2026Article
- Induction of fibrosis in human kidney organoids delineates mechanisms and therapeutic targets of fibrotic kidney disease.Stem cell research & therapy · 2026Article
- Splice modulation of COL4A5 reinstates collagen IV assembly in an organoid model of Alport syndrome.JCI insight · 2026Article
- Personalized Models of Biological Barriers and Their Diseases: Recent Progress with Organs-On-Chips.Advanced biology · 2026Review
- Organoids in Genetic Disorders: from Disease Modeling to Translational Applications.Stem cell reviews and reports · 2025Review
- Kidney Organoids: Current Advances and Applications.Life (Basel, Switzerland) · 2025Review
- The Life of a Kidney Podocyte.Acta physiologica (Oxford, England) · 2025Review
- Nephron progenitor fate is modulated by angiotensin type 1 receptor signaling in human kidney organoids.Stem cells (Dayton, Ohio) · 2025Article
- A kidney organoid-based readout to assess disease activity in primary and recurrent focal segmental glomerulosclerosis.Kidney international · 2025Article
- Advances and Challenges in Modeling Autosomal Dominant Polycystic Kidney Disease: A Focus on Kidney Organoids.Biomedicines · 2025Review
- Organoids: their emerging essential role in pathological mechanisms and drug discovery of diabetes and its complications.Frontiers in pharmacology · 2025Review
- Advancements in theFrontiers in toxicology · 2025Review
- Mouse embryonic kidney transplantation identifies maturation defects in the medulla.Scientific reports · 2024Article
- Investigation of exon skipping therapy in kidney organoids from Alport syndrome patients derived iPSCs.Genes to cells : devoted to molecular & cellular mechanisms · 2024Article
- FromLab on a chip · 2024Article
- Kidney organoids: steps towards better organization and function.Biochemical Society transactions · 2024Review
- Kidney Organoid Modeling of WT1 Mutations Reveals Key Regulatory Paths Underlying Podocyte Development.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- Review
29 more citing papers are in PubMed but not listed here.
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Authors and funding
14 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations in the NPHS1 gene, which encodes NEPHRIN, cause congenital nephrotic syndrome, resulting from impaired slit diaphragm (SD) formation in glomerular podocytes. However, methods for SD reconstitution have been unavailable, thereby limiting studies in the field. In the present study, we established human induced pluripotent stem cells (iPSCs) from a patient with an NPHS1 missense mutation, and reproduced the SD formation process using iPSC-derived kidney organoids. The mutant NEPHRIN failed to become localized on the cell surface for pre-SD domain formation in the induced podocytes. Upon transplantation, the mutant podocytes developed foot processes, but exhibited impaired SD formation. Genetic correction of the single amino acid mutation restored NEPHRIN localization and phosphorylation, colocalization of other SD-associated proteins, and SD formation. Thus, these kidney organoids from patient-derived iPSCs identified SD abnormalities in the podocytes at the initial phase of congenital nephrotic disease.
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