Generation of the integration-free induced pluripotent stem cell line (FHUSTCi001-A) from a patient with glomerulopathy with fibronectin deposits harboring FN1 mutation

• Published in: Stem cell research Vol. 61; p. 102751
• Main Authors: Yu, Xin, Wang, Haitao, Xiao, Fang, Jiang, Shan, Li, Kailin, Chen, Qiuxing, Wang, Gaosheng, Kong, Feng, Zhao, Shengtian
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 01.05.2022; Elsevier
• Subjects: Child; Female; Glomerulonephritis, Membranoproliferative - metabolism; Glomerulonephritis, Membranoproliferative - pathology; Humans; Induced Pluripotent Stem Cells - metabolism; Leukocytes, Mononuclear - pathology; Male; Mutation - genetics
• ISSN: 1873-5061; 1876-7753
• DOI: 10.1016/j.scr.2022.102751

•An iPSC line from a Glomerulopathy with Fibronectin Deposits patient with a heterozygous c.5602G > A (p.V1868M) mutation in the FN1 gene.•Characterization of the GFND patient iPSC line and cultured it over the passage-10.•Confirmation of the pluripotency and cell line identity of this iPSC. Glomerulopathy with fibronectin deposits (GFND) is an autosomal dominant kidney disease exhibiting microscopic hematuria, proteinuria, and hypertension that may lead to end-stage renal failure. In this study, using non-integrative episomal vectors an induced pluripotent stem cell (iPSC) line, FHUSTCi001-A, was derived from peripheral blood mononuclear cells of an 11-year-old boy with GFND carrying a heterozygous c.5602G > A (p.V1868M) mutation in the FN1 gene. The generated iPSC line has a normal karyotype, expresses pluripotency markers, and has the capacity to form all three germ layers in vivo. This iPSC line offers a useful cellular model to study the pathogenesis of GFND disease.