Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children
- 30 June 2010
- journal article
- case report
- Published by Elsevier BV in Molecular Genetics and Metabolism
- Vol. 100 (2), 123-128
- https://doi.org/10.1016/j.ymgme.2010.02.008
Abstract
No abstract availableKeywords
This publication has 17 references indexed in Scilit:
- FIC1-mediated stimulation of FXR activity is decreased with PFIC1 mutations in HepG2 cellsThe Esophagus, 2009
- Progressive familial intrahepatic cholestasisOrphanet Journal of Rare Diseases, 2009
- Short-Chain Ubiquitination Is Associated with the Degradation Rate of a Cell-Surface-Resident Bile Salt Export Pump (BSEP/ABCB11)Published by American Society for Pharmacology & Experimental Therapeutics (ASPET) ,2008
- The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptorHepatology, 2008
- 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumpsHepatology, 2007
- Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growthPediatric Transplantation, 2007
- Two common PFIC2 mutations are associated with the impaired membrane trafficking of BSEP/ABCB11Hepatology, 2005
- Identification of HAX-1 as a Protein That Binds Bile Salt Export Protein and Regulates Its Abundance in the Apical Membrane of Madin-Darby Canine Kidney CellsPublished by Elsevier BV ,2004
- Retarded Intracellular Lipid Transport Associated With Reduced Expression of Cdc42, a Member of Rho-GTPases, in Human Aged Skin FibroblastsArteriosclerosis, Thrombosis, and Vascular Biology, 2002
- The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type IIJCI Insight, 2002