Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.

[1]  Hiroshi Suzuki,et al.  FIC1-mediated stimulation of FXR activity is decreased with PFIC1 mutations in HepG2 cells , 2009, Journal of Gastroenterology.

[2]  E. Jacquemin,et al.  Progressive familial intrahepatic cholestasis , 2009, Orphanet journal of rare diseases.

[3]  Y. Sugiyama,et al.  Short-Chain Ubiquitination Is Associated with the Degradation Rate of a Cell-Surface-Resident Bile Salt Export Pump (BSEP/ABCB11) , 2009, Molecular Pharmacology.

[4]  T. Miloh,et al.  The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor , 2008, Hepatology.

[5]  M. Çakır,et al.  Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth , 2007, Pediatric transplantation.

[6]  Y. Sugiyama,et al.  4‐phenylbutyrate enhances the cell surface expression and the transport capacity of wild‐type and mutated bile salt export pumps , 2007, Hepatology.

[7]  J. Moseley,et al.  Myosin II Regulatory Light Chain Is Required for Trafficking of Bile Salt Export Protein to the Apical Membrane in Madin-Darby Canine Kidney Cells* , 2005, Journal of Biological Chemistry.

[8]  Y. Sugiyama,et al.  Two common PFIC2 mutations are associated with the impaired membrane trafficking of BSEP/ABCB11 , 2005, Hepatology.

[9]  R. Houwen,et al.  Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. , 2004, Gastroenterology.

[10]  J. Moseley,et al.  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 Cells* , 2004, Journal of Biological Chemistry.

[11]  L. Pawlikowska,et al.  Molecular basis of intrahepatic cholestasis , 2004, Annals of medicine.

[12]  K. Faber,et al.  A progressive familial intrahepatic cholestasis type 2 mutation causes an unstable, temperature-sensitive bile salt export pump. , 2004, Journal of hepatology.

[13]  S. Yamashita,et al.  Retarded Intracellular Lipid Transport Associated With Reduced Expression of Cdc42, a Member of Rho-GTPases, in Human Aged Skin Fibroblasts: A Possible Function of Cdc42 in Mediating Intracellular Lipid Transport , 2002, Arteriosclerosis, thrombosis, and vascular biology.

[14]  J. Boyer,et al.  The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type II. , 2002, The Journal of clinical investigation.

[15]  L. Bull.  Hereditary forms of intrahepatic cholestasis. , 2002, Current opinion in genetics & development.

[16]  T. Yorifuji,et al.  Successful living-donor liver transplantation from an asymptomatic carrier mother in ornithine transcarbamylase deficiency. , 2001, The Journal of pediatrics.

[17]  S. Brusilow.  Urea cycle enzymes , 2001 .

[18]  Kouichi R. Tanaka,et al.  Histological improvement in native liver after auxiliary partial liver transplantation for ornithine transcarbamylase deficiency , 1996, The Lancet.