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An in vitro model of polycystic liver disease using genome-edited human inducible pluripotent stem cells.

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An in vitro model of polycystic liver disease using genome-edited human inducible pluripotent stem cells.

Stem Cell Res. 2018 Aug 24;32:17-24

Authors: Kamiya A, Chikada H, Ida K, Ando E, Tsuruya K, Kagawa T, Inagaki Y

Abstract
In the developing liver, bile duct structure is formed through differentiation of hepatic progenitor cells (HPC) into cholangiocytes. A subtype of polycystic liver diseases characterized by uncontrolled expansion of bile ductal cells is caused by genetic abnormalities such as in that of protein kinase C substrate 80 K-H (PRKCSH). In this study, we aimed to mimic the disease process in vitro by genome editing of the PRKCSH locus in human inducible pluripotent stem (iPS) cells. A proportion of cultured human iPS cell-derived CD13+CD133+ HPC differentiated into CD13- cells. During the subsequent gel embedding culture, CD13- cells formed bile ductal marker-positive cystic structures with the polarity of epithelial cells. A deletion of PRKCSH gene increased expression of cholangiocytic transcription factors in CD13- cells and the number of cholangiocytic cyst structure. These results suggest that PRKCSH deficiency promotes the differentiation of HPC-derived cholangiocytes, providing a good in vitro model to analyze the molecular mechanisms underlying polycystic diseases.

PMID: 30172093 [PubMed – as supplied by publisher]

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