A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD)

Markus Moser, Sonja Matthiesen, Jutta Kirfel, Hubert Schorle, Carsten Bergmann, Jan Senderek, Sabine Rudnik‐Schöneborn, Klaus Zerres, Reinhard Buettner – 19 April 2005 – Autosomal recessive polycystic kidney disease (ARPKD) is an important cause of liver‐ and renal‐related morbidity and mortality in childhood. Recently, PKHD1, the gene encoding the transmembrane protein polyductin, was shown to be mutated in ARPKD patients. We here describe the first mouse strain, generated by targeted mutation of Pkhd1.

Percutaneous radiofrequency ablation of hepatocellular carcinoma as a bridge to liver transplantation

David S. K. Lu, Nam C. Yu, Steven S. Raman, Charles Lassman, Myron J. Tong, Carolyn Britten, Francisco Durazo, Sammy Saab, Steven Han, Richard Finn, Jonathan R. Hiatt, Ronald W. Busuttil – 19 April 2005 – Orthotopic liver transplantation (OLT) can be a definitive treatment for patients with hepatocellular carcinoma (HCC). Prolonged waiting times for cadaveric livers, however, may lead to dropout from the waiting list or worsened post‐OLT prognosis as a result of interval tumor progression.

Lipopolysaccharide‐induced tyrosine nitration and inactivation of hepatic glutamine synthetase in the rat

Boris Görg, Matthias Wettstein, Sabine Metzger, Freimut Schliess, Dieter Häussinger – 19 April 2005 – Glutamine synthetase (GS) in the liver is restricted to a small perivenous hepatocyte population and plays an important role in the scavenging of ammonia that has escaped the periportal urea‐synthesizing compartment. We examined the effect of a single intraperitoneal injection of lipopolysaccharide (LPS) in vivo on glutamine synthesis in rat liver.

Oxidative stress stimulates proliferation and invasiveness of hepatic stellate cells via a MMP2‐mediated mechanism

Andrea Galli, Gianluca Svegliati‐Baroni, Elisabetta Ceni, Stefano Milani, Francesco Ridolfi, Renata Salzano, Mirko Tarocchi, Cecilia Grappone, Giulia Pellegrini, Antonio Benedetti, Calogero Surrenti, Alessandro Casini – 19 April 2005 – Experimental evidence indicates that reactive oxygen species (ROS) are involved in the development of hepatic fibrosis; they induce hepatic stellate cells (HSC) proliferation and collagen synthesis.

Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis

Verena Keitel, Martin Burdelski, Ulrich Warskulat, Thomas Kühlkamp, Dietrich Keppler, Dieter Häussinger, Ralf Kubitz – 19 April 2005 – Mutations of the bile salt export pump (BSEP) or the multidrug resistance P‐glycoprotein 3 (MDR3) are linked to impaired bile salt homeostasis and lead to progressive familial intrahepatic cholestasis (PFIC)‐2 and ‐3, respectively. The regulation of bile salt transporters in PFIC is not known.

Systemic infusion of angiotensin II exacerbates liver fibrosis in bile duct–ligated rats

Ramón Bataller, Erwin Gäbele, Christopher J. Parsons, Terry Morris, Liu Yang, Robert Schoonhoven, David A. Brenner, Richard A. Rippe – 19 April 2005 – Recent evidence indicates that the renin–angiotensin system (RAS) plays a major role in liver fibrosis. Here, we investigate whether the circulatory RAS, which is frequently activated in patients with chronic liver disease, contributes to fibrosis progression. To test this hypothesis, we increased circulatory angiotensin II (Ang II) levels in rats undergoing biliary fibrosis.

A chimeric GB virus B with 5′ nontranslated RNA sequence from hepatitis C virus causes hepatitis in tamarins

Rene Rijnbrand, Yan Yang, Lucy Beales, Francis Bodola, Kathryn Goettge, Lisette Cohen, Robert E. Lanford, Stanley M. Lemon, Annette Martin – 19 April 2005 – Only humans and chimpanzees are fully permissive for replication of hepatitis C virus (HCV), an important cause of liver cirrhosis and cancer worldwide. The absence of suitable animal models limits opportunities for in vivo evaluation of candidate hepatitis C therapeutics and slows progress in the field.

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