Polycystic disease of the liver

Gregory T. Everson, Matthew R. G. Taylor, R. Brian Doctor – 7 March 2007 – Autosomal dominant polycystic disease is genetically heterogeneous with mutations in two distinct genes predisposing to the combination of renal and liver cysts (AD‐PKD1 and AD‐PKD2) and mutations in a third gene yielding isolated liver cysts (the polycystic liver disease gene). Transcription and translation of the PKD1 gene produces polycystin‐1, an integral membrane protein that may serve as an extracellular receptor.

Myeloperoxidase‐positive inflammatory cells participate in bile duct damage in primary biliary cirrhosis through nitric oxide‐mediated reactions

Chih‐Te Wu, Jason P. Eiserich, Aftab A. Ansari, Ross L. Coppel, Sripriya Balasubramanian, Christopher L. Bowlus, M. Eric Gershwin, Judy Van De Water – 7 March 2007 – Previous studies have suggested that increased nitric oxide (NO)‐mediated products are found in the livers of subjects with primary biliary cirrhosis (PBC), but the mechanisms involved remain enigmatic.

Characterization of premature liver polyploidy in DNA repair (Ercc1)‐deficient mice

Michael D. Chipchase, Mary O'Neill, David W. Melton – 7 March 2007 – ERCC1‐XPF is the endonuclease that cuts 5′ of the damage in nucleotide excision repair (NER). Unlike other NER proteins, ERCC1‐XPF is also involved in recombination and the repair of DNA interstrand cross‐links. Unique among the NER gene knockouts, Ercc1 null mice are severely runted with high levels of hepatocyte polyploidy. To understand the link between DNA repair deficiency and polyploidy we have compared the premature polyploidy in Ercc1 null liver with the normal development of polyploidy in aging control mice.

Human hepatic stellate cells show features of antigen‐presenting cells and stimulate lymphocyte proliferation

Odette Viñas, Ramón Bataller, Pau Sancho‐Bru, Pere Ginès, Cristina Berenguer, Carlos Enrich, Josep M. Nicolás, Guadalupe Ercilla, Teresa Gallart, Jordi Vives, Vicente Arroyo, Juan Rodés – 7 March 2007 – Following cell activation, hepatic stellate cells (HSCs) acquire proinflammatory and profibrogenic properties. We investigated whether activated HSCs also display immune properties.

Characterization of recombinant monoclonal IgA anti—PDC‐E2 autoantibodies derived from patients with PBC

Nobuyoshi Fukushima, Greg Nalbandian, Judy Van De Water, Kandra White, Aftab A. Ansari, Patrick Leung, Thomas Kenny, Shizuo G. Kamita, Bruce D. Hammock, Ross L. Coppel, Freida Stevenson, Hiromi Ishibashi, M. Eric Gershwin – 7 March 2007 – Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by the presence of autoantibodies to mitochondria (AMA). Recent evidence suggests that PBC develops after a locally driven response in the mucosa, where immunoglobulin A (IgA) is the dominant antibody isotype.

Peptide antibiotic human beta‐defensin‐1 and −2 contribute to antimicrobial defense of the intrahepatic biliary tree

Kenichi Harada, Kazuo Ohba, Satoru Ozaki, Kumiko Isse, Toshiya Hirayama, Akihiro Wada, Yasuni Nakanuma – 7 March 2007 – Human beta‐defensins (hBDs) are important antimicrobial peptides that contribute to innate immunity at mucosal surfaces. This study was undertaken to investigate the expression of hBD‐1 and hBD‐2 in intrahepatic biliary epithelial cells in specimens of human liver, and 4 cultured cell lines (2 consisting of biliary epithelial cells and 2 cholangiocarcinoma cells). In addition, hBD‐1 and hBD‐2 were assayed in specimens of bile.

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