http://publicationslist.org/data/pberghe/atom.xml Peter V.e. Berghehttp://publicationslist.org/pberghe$basepathfavicon.icoRecent additions to Peter V.e. Berghe's PublicationsList.org pagehttp://publicationslist.org/publications.png2010-03-16T13:30:06Z http://publicationslist.org/pberghe/refid2 2010-03-16T13:11:34Z

Deficiency in P-type ATP8B1 is a severe and clinically highly variable hereditary disorder that is primarily characterized by intrahepatic cholestasis. It presents either as a progressive (progressive familial intrahepatic cholestasis type 1 [PFIC1]) or intermittent (benign recurrent intrahepatic cholestasis type 1 [BRIC1]) disease. ATP8B1 deficiency is caused by autosomal recessive mutations in t...<br/><br/>Lieke M van der Velden, Janneke M Stapelbroek, Elmar Krieger, Peter V E van den Berghe, Ruud Berger, Patricia M Verhulst, Joost C M Holthuis, Roderick H J Houwen, Leo W J Klomp, Stan F J van de Graaf (2010) <i>Hepatology</i> <i></i> <i></i> 51: 1 286-296<br/>

http://publicationslist.org/pberghe/refid4 2010-03-16T13:11:34Z

Copper is an essential but potentially harmful trace element involved in many enzymatic processes that require redox chemistry. Cellular copper homeostasis in mammals is predominantly maintained by posttranslational regulation of copper import and export through the copper import proteins hCTR1 and hCTR2 and the copper exporters ATP7A and ATP7B. Regulation of copper uptake and export is achieved b...<br/><br/>Peter V E van den Berghe, Leo W J Klomp (2010) <i>J Biol Inorg Chem</i> <i></i> <i></i> 15: 1 37-46<br/>

http://publicationslist.org/pberghe/refid3 2010-03-16T13:11:34Z

The transition metal copper is an essential trace element involved in many enzymatic processes that require redox-chemistry. The redox-activity of copper is potentially harmful. Severe imbalance of copper homeostasis can occur with some hereditary disorders of copper metabolism. Copper is acquired from the diet by intestinal absorption and is subsequently distributed throughout the body. The regul...<br/><br/>Peter V E van den Berghe, Leo W J Klomp (2009) <i>Nutr Rev</i> <i></i> <i></i> 67: 11 658-672<br/>

http://publicationslist.org/pberghe/refid1 2010-03-16T13:11:34Z

Wilson disease (WD) is an autosomal recessive copper overload disorder of the liver and basal ganglia. WD is caused by mutations in the gene encoding ATP7B, a protein localized to the trans-Golgi network that primarily facilitates hepatic copper excretion. Current treatment comprises reduction of circulating copper by zinc supplementation or copper chelation. Despite treatment, a significant numbe...<br/><br/>Peter V E van den Berghe, Janneke M Stapelbroek, Elmar Krieger, Prim de Bie, Stan F J van de Graaf, Reinoud E A de Groot, Ellen van Beurden, Ellen Spijker, Roderick H J Houwen, Ruud Berger, Leo W J Klomp (2009) <i>Hepatology</i> <i></i> <i></i> 50: 6 1783-1795<br/>

http://publicationslist.org/pberghe/refid5 2010-03-16T13:11:34Z

Filamin A, or actin-binding protein 280, is a ubiquitously expressed cytosolic protein that interacts with intracellular domains of multiple receptors to control their subcellular distribution, and signaling capacity. In this study, we document interaction between FcgammaRI, a high-affinity IgG receptor, and filamin A by yeast two-hybrid techniques and coimmunoprecipitation. Both proteins colocali...<br/><br/>Jeffrey M Beekman, Cees E van der Poel, Joke A van der Linden, Debbie L C van den Berg, Peter V E van den Berghe, Jan G J van de Winkel, Jeanette H W Leusen (2008) <i>J Immunol</i> <i></i> <i></i> 180: 6 3938-3945<br/>

http://publicationslist.org/pberghe/refid7 2010-03-16T13:11:34Z

High-affinity cellular copper uptake is mediated by the CTR (copper transporter) 1 family of proteins. The highly homologous hCTR (human CTR) 2 protein has been identified, but its function in copper uptake is currently unknown. To characterize the role of hCTR2 in copper homoeostasis, epitope-tagged hCTR2 was transiently expressed in different cell lines. hCTR2-vsvG (vesicular-stomatitis-virus gl...<br/><br/>Peter V E van den Berghe, Dineke E Folmer, Helga E M Malingré, Ellen van Beurden, Adriana E M Klomp, Bart van de Sluis, Maarten Merkx, Ruud Berger, Leo W J Klomp (2007) <i>Biochem J</i> <i></i> <i></i> 407: 1 49-59<br/>

http://publicationslist.org/pberghe/refid6 2010-03-16T13:11:34Z

BACKGROUND & AIMS: Wilson's disease (WD) is characterized by hepatic copper overload and caused by mutations in the gene encoding the copper-transporting P-type adenosine triphosphatase (ATPase) ATP7B. ATP7B interacts with COMMD1, a protein that is deleted in Bedlington terriers with hereditary copper toxicosis. Here we characterized the implications of the interaction between COMMD1 and ATP7B in ...<br/><br/>Prim de Bie, Bart van de Sluis, Ezra Burstein, Peter V E van de Berghe, Patricia Muller, Ruud Berger, Jonathan D Gitlin, Cisca Wijmenga, Leo W J Klomp (2007) <i>Gastroenterology</i> <i></i> <i></i> 133: 4 1316-1326<br/>

http://publicationslist.org/pberghe/refid8 2010-03-16T13:11:34Z

RNA-mediated interference (RNAi) is a method to inhibit gene function by introduction of double-stranded RNA (dsRNA). Recently, an RNAi library was constructed that consists of bacterial clones expressing dsRNA, corresponding to nearly 90% of the 19,427 predicted genes of C. elegans. Feeding of this RNAi library to the standard wild-type laboratory strain Bristol N2 detected phenotypes for approxi...<br/><br/>Femke Simmer, Celine Moorman, Alexander M van der Linden, Ewart Kuijk, Peter V E van den Berghe, Ravi S Kamath, Andrew G Fraser, Julie Ahringer, Ronald H A Plasterk (2003) <i>PLoS Biol</i> <i></i> <i></i> 1: 1 <br/>