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Generation of special autosomal dominant polycystic kidney disease iPSCs with the capability of functional kidney-like cell differentiation.


ABSTRACT:

Background

Human induced pluripotent stem cells (iPSCs) have been verified as a powerful cell model for the study of pathogenesis in hereditary disease. Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations of PKD or non-PKD genes. The pathogenesis of ADPKD remains unexplored because of the lack of a true human cell model.

Methods

Six ADPKD patients and four healthy individuals were recruited as donors of somatic cells from a Chinese ADPKD family without mutations of the PKD genes but carrying SAMSN1 gene deletion. The ADPKD-iPSCs were generated from somatic cells and were induced into kidney-like cells (KLCs) by a novel three-step method involving cytokines and renal epithelium growth medium. Furthermore, we analyzed functional properties of these KLCs

SUBMITTER: Huang J 

PROVIDER: S-EPMC5606115 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature