Search Results - "Paulusma, Coen C."

Therapeutic base editing in the adult liver by Paulusma, Coen C., Bosma, Piter J.

“…Gene editing to correct inherited liver disorders has promise for future therapeutic intervention, but lack of effective and safe delivery of the gene-editing…”
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A biliary HCO3− umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes by Hohenester, Simon, Maillette de Buy Wenniger, Lucas, Paulusma, Coen C., van Vliet, Sandra J., Jefferson, Douglas M., Oude Elferink, Ronald P., Beuers, Ulrich

“…Human cholangiocytes are continuously exposed to millimolar levels of hydrophobic bile salt monomers. We recently hypothesized that an apical biliary HCO 3−…”
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Sodium taurocholate cotransporting polypeptide (SLC10A1) deficiency: Conjugated hypercholanemia without a clear clinical phenotype by Vaz, Frédéric M., Paulusma, Coen C., Huidekoper, Hidde, de Ru, Minke, Lim, Cynthia, Koster, Janet, Ho‐Mok, Kam, Bootsma, Albert H., Groen, Albert K., Schaap, Frank G., Oude Elferink, Ronald P. J., Waterham, Hans R., Wanders, Ronald J.A.

“…The enterohepatic circulation of bile salts is an important physiological route to recycle bile salts and ensure intestinal absorption of dietary lipids. The…”
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CDC50A is required for aminophospholipid transport and cell fusion in mouse C2C12 myoblasts by Grifell-Junyent, Marta, Baum, Julia F, Välimets, Silja, Herrmann, Andreas, Paulusma, Coen C, López-Marqués, Rosa L, Günther Pomorski, Thomas

“…Myoblast fusion is essential for the formation of multinucleated muscle fibers and is promoted by transient changes in the plasma membrane lipid distribution…”
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Bile salt signaling and bile salt-based therapies in cardiometabolic disease by Groenen, Claire C J, Nguyen, Thuc-Anh, Paulusma, Coen C, van de Graaf, Stan F J

“…Bile salts have an established role in the emulsification and intestinal absorption of dietary lipids, and their homeostasis is tightly controlled by various…”
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Soluble Adenylyl Cyclase Regulates Bile Salt‐Induced Apoptosis in Human Cholangiocytes by Chang, Jung‐Chin, Go, Simei, de Waart, Dirk R., Munoz‐Garrido, Patricia, Beuers, Ulrich, Paulusma, Coen C., Oude Elferink, Ronald

“…Anion exchanger 2 (AE2), the principal bicarbonate secretor in the human biliary tree, is down‐regulated in primary biliary cholangitis. AE2 creates a…”
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Liver-directed gene therapy results in long-term correction of progressive familial intrahepatic cholestasis type 3 in mice by Aronson, Sem J., Bakker, Robert S., Shi, Xiaoxia, Duijst, Suzanne, ten Bloemendaal, Lysbeth, de Waart, Dirk R., Verheij, Joanne, Ronzitti, Giuseppe, Oude Elferink, Ronald P., Beuers, Ulrich, Paulusma, Coen C., Bosma, Piter J.

“…[Display omitted] •Adeno-associated virus (AAV)-mediated gene therapy can correct Abcb4 deficiency (PFIC3) in mice.•By restoring phospholipid transport to…”
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The P4-ATPase ATP9A is a novel determinant of exosome release by Naik, Jyoti, Hau, Chi M, Ten Bloemendaal, Lysbeth, Mok, Kam S, Hajji, Najat, Wehman, Ann M, Meisner, Sander, Muncan, Vanesa, Paauw, Nanne J, de Vries, H E, Nieuwland, Rienk, Paulusma, Coen C, Bosma, Piter J

“…Extracellular vesicles (EVs) released by cells have a role in intercellular communication to regulate a wide range of biological processes. Two types of EVs…”
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Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein) by Oude Elferink, Ronald P J, Paulusma, Coen C

“…Like several other ATP-binding cassette (ABC) transporters, ABCB4 is a lipid translocator. It translocates phosphatidylcholine (PC) from the inner to the outer…”
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P4 ATPases: flippases in health and disease by van der Mark, Vincent A, Elferink, Ronald P J Oude, Paulusma, Coen C

“…P4 ATPases catalyze the translocation of phospholipids from the exoplasmic to the cytosolic leaflet of biological membranes, a process termed "lipid flipping"…”
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Vps33b is crucial for structural and functional hepatocyte polarity by Hanley, Joanna, Dhar, Dipok Kumar, Mazzacuva, Francesca, Fiadeiro, Rebeca, Burden, Jemima J, Lyne, Anne-Marie, Smith, Holly, Straatman-Iwanowska, Anna, Banushi, Blerida, Virasami, Alex, Mills, Kevin, Lemaigre, Frédéric P, Knisely, A.S, Howe, Steven, Sebire, Neil, Waddington, Simon N, Paulusma, Coen C, Clayton, Peter, Gissen, Paul

“…Graphical abstract…”
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Complementary Functions of the Flippase ATP8B1 and the Floppase ABCB4 in Maintaining Canalicular Membrane Integrity by Groen, Annemiek, Romero, Marta Rodriguez, Kunne, Cindy, Hoosdally, Sarah J, Dixon, Peter H, Wooding, Carol, Williamson, Catherine, Seppen, Jurgen, van den Oever, Karin, Mok, Kam S, Paulusma, Coen C, Linton, Kenneth J, Oude Elferink, Ronald P.J

“…Background & Aims Progressive familial intrahepatic cholestasis can be caused by mutations in ABCB4 or ATP8B1; each encodes a protein that translocates…”
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ATP8B1 Deficiency Results in Elevated Mitochondrial Phosphatidylethanolamine Levels and Increased Mitochondrial Oxidative Phosphorylation in Human Hepatoma Cells by Gómez-Mellado, Valentina E., Chang, Jung-Chin, Ho-Mok, Kam S., Bernardino Morcillo, Carmen, Kersten, Remco H. J., Oude Elferink, Ronald P. J., Verhoeven, Arthur J., Paulusma, Coen C.

“…ATP8B1 is a phospholipid flippase that is deficient in patients with progressive familial intrahepatic cholestasis type 1 (PFIC1). PFIC1 patients suffer from…”
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Phospholipid flippases attenuate LPS-induced TLR4 signaling by mediating endocytic retrieval of Toll-like receptor 4 by van der Mark, Vincent A., Ghiboub, Mohammed, Marsman, Casper, Zhao, Jing, van Dijk, Remco, Hiralall, Johan K., Ho-Mok, Kam S., Castricum, Zoë, de Jonge, Wouter J., Oude Elferink, Ronald P. J., Paulusma, Coen C.

“…P4-ATPases are lipid flippases that catalyze the transport of phospholipids to create membrane phospholipid asymmetry and to initiate the biogenesis of…”
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Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis by van der Woerd, Wendy L, Wichers, Catharina G.K, Vestergaard, Anna L, Andersen, Jens Peter, Paulusma, Coen C, Houwen, Roderick H.J, van de Graaf, Stan F.J

“…Graphical abstract…”
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Glycochenodeoxycholate Promotes Liver Fibrosis in Mice with Hepatocellular Cholestasis by Hohenester, Simon, Kanitz, Veronika, Kremer, Andreas E, Paulusma, Coen C, Wimmer, Ralf, Kuehn, Helen, Denk, Gerald, Horst, David, Elferink, Ronald Oude, Beuers, Ulrich

“…Hydrophobic bile salts are considered to promote liver fibrosis in cholestasis. However, evidence for this widely accepted hypothesis remains scarce. In…”
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P4 ATPases – The physiological relevance of lipid flipping transporters by Paulusma, Coen C., Oude Elferink, Ronald P.J.

“…P4 ATPases are integral transmembrane proteins implicated in phospholipid translocation from the exoplasmic to the cytosolic leaflet of biological membranes…”
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ATP11C targets basolateral bile salt transporter proteins in mouse central hepatocytes by de Waart, Dirk R., Naik, Jyoti, Utsunomiya, Karina S., Duijst, Suzanne, Ho‐Mok, Kam, Bolier, A. Ruth, Hiralall, Johan, Bull, Laura N., Bosma, Piter J., Oude Elferink, Ronald P.J., Paulusma, Coen C.

“…ATP11C is a homolog of ATP8B1, both of which catalyze the transport of phospholipids in biological membranes. Mutations in ATP8B1 cause progressive familial…”
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Activity of the Bile Salt Export Pump (ABCB11) Is Critically Dependent on Canalicular Membrane Cholesterol Content by Paulusma, Coen C., de Waart, D.Rudi, Kunne, Cindy, Mok, Kam S., Elferink, Ronald P.J.Oude

“…Mutations in ATP8B1 cause severe inherited liver disease. The disease is characterized by impaired biliary bile salt excretion (cholestasis), but the mechanism…”
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Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport by Paulusma, Coen C., Groen, Annemiek, Kunne, Cindy, Ho‐Mok, Kam S., Spijkerboer, Astrid L., Rudi de Waart, D., Hoek, Frans J., Vreeling, Heleen, Hoeben, Kees A., van Marle, Jan, Pawlikowska, Ludmila, Bull, Laura N., Hofmann, Alan F., Knisely, A. S., Oude Elferink, Ronald P. J.

“…Progressive familial intrahepatic cholestasis type 1 (PFIC1, Byler disease, OMIM 211600) is a severe inherited liver disease caused by mutations in ATP8B1…”
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