Pluripotent stem cell-derived bile canaliculi-forming hepatocytes to study genetic liver diseases involving hepatocyte polarity
Summary
This study demonstrates that cells that are isolated from urine can be reprogrammed in a dish towards hepatocytes that display architectural characteristics similar to those seen in the intact liver. The application of this methodology to cells from patients diagnosed with inherited copper metabolism-related liver diseases (that is, Wilson disease and MEDNIK syndrome) revealed unexpected and novel insights into patient mutation-specific disease mechanisms and drug responses. Copyright © 2019 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
| Authors | Overeem AW, Klappe K, Parisi S, Klöters-Planchy P, Mataković L, du Teil Espina M, Drouin CA, Weiss KH, van IJzendoorn SCD |
|---|---|
| Journal | Journal of hepatology |
| Publication Date | 2019 Aug;71(2):344-356 |
| PubMed | 30965071 |
| DOI | 10.1016/j.jhep.2019.03.031 |