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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