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Cholangiopathy and Biliary Fibrosis in Cyp2c70-Deficient Mice Are Fully Reversed by Ursodeoxycholic Acid

10 December 2020

Jan Freark de Boer, Hilde D. de Vries, Anna Palmiotti, Rumei Li, Marwah Doestzada, Joanne A. Hoogerland, Jingyuan Fu, Anouk M. La Rose, Marit Westerterp, Niels L. Mulder, Milaine V. Hovingh, Martijn Koehorst, Niels J. Kloosterhuis, Justina C. Wolters, Vincent W. Bloks, Joel T. Haas, David Dombrowicz, Bart Staels, Bart van de Sluis, Folkert Kuipers

We have generated and characterized a Cyp2c70-deficient mouse model having a human-like bile acid (BA) composition. Both genders show transient neonatal cholestasis, but only females develop considerable pathologic features with age. Addition of hydrophilic bile acid ursodeoxycholic acid  (UDCA) to the diet completely normalized all hepatic functions and fully restored liver morphology of  female Cyp2c70-deficient mice.

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