Personalized disease recurrence modeling using iPSC-derived podocytes in patients with idiopathic nephrotic syndrome.

ABSTRACT:

Background

Primary focal segmental glomerulosclerosis (FSGS) is characterized by podocyte injury and treatment-resistant nephrotic syndrome. Recurrence of the original disease after kidney transplantation (rFSGS) occurs in 10%-50% of patients. Unidentified circulating permeability factors (CPF) are likely involved in FSGS pathogenesis. We hypothesized that donor podocyte susceptibility to CPF is also relevant. We developed a personalized model for (r)FSGS using induced pluripotent stem cell (iPSC)-derived podocytes from patients and kidney donors.

Methods

Five patients and their respective living kidney donors were included. Three patients had developed rFSGS, and two patients manifested no symptoms of rFSGS. One patient (P5) had heterozygous mutations in NPHS2. Peripheral b

SUBMITTER: van den Berge BT 

PROVIDER: S-EPMC12451694 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature