Related Experiment Video
Updated: Dec 22, 2025

Author Spotlight: Optimizing iPSC Differentiation for Efficient Production to Generate Kidney Organoids
Published on: September 1, 2023
Generation of two human induced pluripotent stem cell lines derived from two juvenile nephronophthisis patients with
Yutaka Arai1, Miho Takami2, Yuri An2
1iPS Cell Advanced Characterization and Development Team, BioResource Research Center, RIKEN, 3-1-1 Koyadai, Tsukuba, Ibaraki 305-0074, Japan; Department of Molecular Pharmacology, Graduate School of Pharmaceutical Sciences and Faculty of Pharmaceutical Sciences, Tokyo University of Science, 2641 Yamazaki, Noda, Chiba, 278-8510, Japan.
Abstract:
Juvenile nephronophthisis is an inherited renal ciliopathy, causing cystic kidney disease, renal fibrosis, and end-stage renal failure. Human induced pluripotent stem cell (hiPSC) lines, derived from two Juvenile nephronophthisis patients, were generated from peripheral blood mononuclear cells by episomal plasmid vectors. Generated hiPSC lines showed self-renewal and pluripotency and carried a large deletion in NPHP1 (Nephrocystin 1) gene. Since the molecular pathogenesis caused by NPHP1 dysfunction remains unclear, these cell resources provide useful tools to establish disease models and to develop new therapies for juvenile nephronophthisis.
Related Concept Videos
iPS Cell Differentiation
EPS and iPS Cells in Disease Research
Induced Pluripotent Stem Cells
Induced Pluripotent Stem Cells
Somatic...

