Bile acid treatment alters hepatic disease and bile acid transport in peroxisome‐deficient PEX2 Zellweger mice
Megan H. Keane, Henk Overmars, Thomas M. Wikander, Sacha Ferdinandusse, Marinus Duran, Ronald J. A. Wanders, Phyllis L. Faust – 28 March 2007 – The marked deficiency of peroxisomal organelle assembly in the PEX2−/− mouse model for Zellweger syndrome provides a unique opportunity to developmentally and biochemically characterize hepatic disease progression and bile acid products. The postnatal survival of homozygous mutants enabled us to evaluate the response to bile acid replenishment in this disease state.