Research Topics
Genomes and Genes | C C PaulusmaSummaryAffiliation: Academic Medical Center Country: The Netherlands Publications
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Publications
Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol contentCoen C Paulusma
AMC Liver Center, Academic Medical Center, Meibergdreef 69 71, 1105 BK, Amsterdam, The Netherlands
J Biol Chem 284:9947-54. 2009....
Alteration of viral lipid composition by expression of the phospholipid floppase ABCB4 reduces HIV vector infectivityNiek P van Til
AMC Liver Center, Meibergdreef 69, 1105 BK, Amsterdam, The Netherlands
Retrovirology 5:14. 2008..This property enabled us to modulate the lipid composition of HIV vectors and study the effects on membrane composition and infection efficiency...
P4 ATPases--the physiological relevance of lipid flipping transportersCoen C Paulusma
Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, Amsterdam, The Netherlands
FEBS Lett 584:2708-16. 2010..This review highlights the current status of a slowly emerging research field and emphasizes the contribution of P4 ATPases to the vesicle-generating machinery...
ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activityCoen C Paulusma
AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands
Hepatology 47:268-78. 2008..In the plasma membrane, ATP8B1 functions as a flippase for phosphatidylserine. Finally, CDC50A may be the potential beta-subunit or chaperone for ATP8B1 in hepatocytes...
Diseases of intramembranous lipid transportCoen C Paulusma
Amsterdam Liver Center, Department of Experimental Hepatology, Academic Medical Center, Meibergdreef 69 71, S 1 168, 1105 BK Amsterdam, The Netherlands
FEBS Lett 580:5500-9. 2006..In order to elucidate the molecular mechanisms that underlie these disorders, the combination of in vivo, biochemical, and structural analyses on intramembrane transporters is crucial...
Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transportCoen C Paulusma
Amsterdam Liver Center, Department of Experimental Hepatology, Academic Medical Center, Amsterdam, The Netherlands
Hepatology 44:195-204. 2006..The loss of phospholipid asymmetry may subsequently impair bile salt transport and cause cholestasis...
The type 4 subfamily of P-type ATPases, putative aminophospholipid translocases with a role in human diseaseC C Paulusma
Department of Experimental Hepatology, Academic Medical Center AMC Liver Center, Meibergdreef 69 71, 1105 BK Amsterdam, The Netherlands
Biochim Biophys Acta 1741:11-24. 2005....
A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndromeC C Paulusma
Department of Gastrointestinal and Liver Diseases, Center for Liver and Intestinal Research, Academic Medical Center, Amsterdam, The Netherlands
Hepatology 25:1539-42. 1997..In the present study, we have isolated the human homologue of rat cmoat, human cMOAT, and analyzed the corresponding cDNA from fibroblasts of a DJS patient for mutations. Our results show that a mutation in this gene is the cause of DJS...
The canalicular multispecific organic anion transporter and conjugated hyperbilirubinemia in rat and manC C Paulusma
Department of Gastrointestinal and Liver Diseases, Center for Liver and Intestinal Research, Academic Medical Center, Amsterdam, The Netherlands
J Mol Med 75:420-8. 1997..A mutation in the cMOAT gene is responsible for the phenotype observed in TR- rats. This information should soon lead tc a complete genetic characterization of the human Dubin-Johnson syndrome...
Congenital jaundice in rats with a mutation in a multidrug resistance-associated protein geneC C Paulusma
Department of Gastrointestinal and Liver Diseases, Center for Liver and Intestinal Research, Academic Medical Center, Amsterdam, Netherlands
Science 271:1126-8. 1996..In the TR(-) rat, a single-nucleotide deletion in this gene resulted in a reduced messenger RNA level and absence of the protein. It is likely that this mutation accounts for the TR(-) phenotype...
Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein)Ronald P J Oude Elferink
AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands
Pflugers Arch 453:601-10. 2007..In this review, we will discuss the functional aspects of ABCB4 and the regulation of its expression. Furthermore, we will describe the clinical and biochemical consequences of complete and partial deficiency of ABCB4 function...
Multidrug resistance associated protein 2 mediates transport of prostaglandin E2Dirk R de Waart
Department of Experimental Hepatology, Academic Medical Center, Amsterdam, The Netherlands
Liver Int 26:362-8. 2006..Biliary excretion of PGE(2) is also a means of elimination from the liver. We investigated the role of multidrug resistance-associated protein 2 (MRP2) in the transport of PGE(2)...
Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseasesRonald P J Oude Elferink
AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands
Gastroenterology 130:908-25. 2006..In this review we only briefly discuss the inherited defects in transporter function, and we focus on the pathophysiologic concepts that these diseases have generated...
Intestinal bile salt absorption in Atp8b1 deficient miceAnnemiek Groen
AMC Liver Center, Academic Medical Center, Room S1 166, Meibergdreef 69 71, 1105 BK Amsterdam, The Netherlands
J Hepatol 47:114-22. 2007..Despite these findings, we observed only mildly impaired canalicular BS transport. In the present report we tested the hypothesis that Atp8b1(G308V/G308V) mice hyperabsorb BS in the intestine during BS feeding...
Abcg5/8 independent biliary cholesterol excretion in Atp8b1-deficient miceAnnemiek Groen
AMC Liver Center, Academic Medical Center, Amsterdam, The Netherlands
Gastroenterology 134:2091-100. 2008..We therefore studied the relation between Abcg5/Abcg8 expression and biliary cholesterol excretion in mice lacking Atp8b1, Abcg8, or both (GF mice)...
