doi.org

Hepatology

Steatohepatitis/Metabolic Liver Disease

Reduced expression of ATP7B affected by Wilson disease–causing mutations is rescued by pharmacological folding chaperones 4‐phenylbutyrate and curcumin†

van den Berghe, Peter V. E.1; Stapelbroek, Janneke M.1,2; Krieger, Elmar3; de Bie, Prim1,4; van de Graaf, Stan F. J.1; de Groot, Reinoud E. A.1; van Beurden, Ellen1; Spijker, Ellen1; Houwen, Roderick H. J.2; Berger, Ruud1; Klomp, Leo W. J.*,1,‡

1Department of Metabolic and Endocrine Diseases, University Medical Center Utrecht, and the Netherlands Metabolomics Center, Utrecht, The Netherlands

2Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands

3Centre for Molecular and Biomolecular Informatics, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

4Cancer and Vascular Biology Research Center, The Rappaport Faculty of Medicine and Research Institute, Technion‐Israel Institute of Technology, Haifa, Israel

Department of Metabolic and Endocrine Diseases, University Medical Center Utrecht, Lundlaan 6 (KC‐02.069.1), 3584 EA Utrecht, The Netherlands===

Received January 13, 2009

Accepted July 23, 2009

Copyright © 2009 American Association for the Study of Liver Diseases.