A new Mdr2(-/-) mouse model of sclerosing cholangitis with rapid fibrosis progression, early-onset portal hypertension, and liver cancer.

No abstract is available for this paper.

• Publication year

  2015

• Venue

  American Journal of Pathology

• Publication date

  2015-02-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.ajpath.2014.10.013PMID 25478810
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Mouse model of primary biliary cirrhosis with progressive fibrosis: Are we there yet?

  2013cited by this paper
• Therapy for Fibrotic Diseases: Nearing the Starting Line

  2013cited by this paper
• Assessing portal hypertension in liver diseases

  2013cited by this paper
• Genome-wide association study identifies variants associated with progression of liver fibrosis from HCV infection.

  2012cited by this paper
• Tissue transglutaminase does not affect fibrotic matrix stability or regression of liver fibrosis in mice.

  2011cited by this paper
• Therapy for hepatic fibrosis: revisiting the preclinical models.

  2011cited by this paper
• Macrophage-mediated phagocytosis of apoptotic cholangiocytes contributes to reversal of experimental biliary fibrosis.

  2010cited by this paper
• Bone marrow transplantation demonstrates medullar origin of CD34+ fibrocytes and ameliorates hepatic fibrosis in Abcb4−/− mice

  2010cited by this paper
• Evolving challenges in hepatic fibrosis

  2010cited by this paper
• Hepatic venous pressure gradient predicts development of hepatocellular carcinoma independently of severity of cirrhosis.

  2009cited by this paper
• Pharmacological inhibition of integrin αvβ3 aggravates experimental liver fibrosis and suppresses hepatic angiogenesis

  2009cited by this paper
• Liver diseases related to MDR3 (ABCB4) gene deficiency.

  2009cited by this paper
• Liver cirrhosis.

  2008cited by this paper
• Integrin alphavbeta6 is a marker of the progression of biliary and portal liver fibrosis and a novel target for antifibrotic therapies.

  2008cited by this paper
• ABCB4 heterozygous gene mutations associated with fibrosing cholestatic liver disease in adults.

  2008cited by this paper
• Mechanisms of hepatic fibrogenesis.

  2008cited by this paper
• MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes.

  2007cited by this paper
• Pharmacological Reduction of Portal Pressure and Long-Term Risk of First Variceal Bleeding in Patients with Cirrhosis

  2006cited by this paper
• Changes of the hepatic proteome in murine models for toxically induced fibrogenesis and sclerosing cholangitis

  2006cited by this paper
• Differential Susceptibility of BALB/c and BALB/cBy Mice to Graves' Hyperthyroidism

  2006cited by this paper
• Liver fibrosis.

  2005cited by this paper
• Mdr2 (Abcb4)-/- mice spontaneously develop severe biliary fibrosis via massive dysregulation of pro- and antifibrogenic genes.

  2005cited by this paper
• Complement factor 5 is a quantitative trait gene that modifies liver fibrogenesis in mice and humans

  2005cited by this paper
• Spontaneous recovery from micronodular cirrhosis: evidence for incomplete resolution associated with matrix cross-linking.

  2004cited by this paper
• Maintenance of hemodynamic response to treatment for portal hypertension and influence on complications of cirrhosis.

  2004cited by this paper
• Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 (Abcb4) knockout mice.

  2004cited by this paper
• Effects of periadolescent ethanol exposure on alcohol preference in two BALB substrains.

  2004cited by this paper
• Spontaneous cholecysto‐ and hepatolithiasis in Mdr2−/− mice: A model for low phospholipid‐associated cholelithiasis

  2004cited by this paper
• Hemodynamic response to pharmacological treatment of portal hypertension and long‐term prognosis of cirrhosis

  2003cited by this paper
• Ursodeoxycholic acid aggravates bile infarcts in bile duct-ligated and Mdr2 knockout mice via disruption of cholangioles.

  2002cited by this paper
• Genome-wide analysis of hepatic fibrosis in inbred mice identifies the susceptibility locus Hfib1 on chromosome 15.

  2002cited by this paper
• [Mechanisms of hepatic fibrogenesis].

  2002cited by this paper
• [Primary sclerosing cholangitis].

  2000influential reference
• Primary sclerosing cholangitis

  1999cited by this paper
• The usefulness of the reticulin stain in the differential diagnosis of liver nodules on fine-needle aspiration biopsy cell block preparations.

  1997cited by this paper
• Strain-specific differences in mouse hepatic wound healing are mediated by divergent T helper cytokine responses.

  1997cited by this paper
• The role of bile salt composition in liver pathology of mdr2 (-/-) mice: differences between males and females.

  1997cited by this paper
• Effects of Ursodeoxycholate and cholate feeding on liver disease in FVB mice with a disrupted mdr2 P-glycoprotein gene.

  1996cited by this paper
• Mice with homozygous disruption of the mdr2 P-glycoprotein gene. A novel animal model for studies of nonsuppurative inflammatory cholangitis and hepatocarcinogenesis.

  1994cited by this paper
• Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease.

  1993cited by this paper
• Proposal for Standardized Criteria for the Diagnosis of Benign, Borderline, and Malignant Hepatocellular Lesions Arising in Chronic Advanced Liver Disease

  1993cited by this paper
• Transforming growth factors beta 1 and beta 2 are differentially expressed in fibrotic liver disease.

  1991cited by this paper
• [Liver cirrhosis].

  1989cited by this paper

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  2026cites this paper
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  2026cites this paper
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  2026cites this paper
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  2024cites this paper
• Transposon-based oncogene integration in Abcb4(Mdr2)-/- mice recapitulates high susceptibility to cholangiocarcinoma in primary sclerosing cholangitis.

  2024cites this paper
• Oral N-acetylcysteine ameliorates liver fibrosis and enhances regenerative responses in Mdr2 knockout mice

  2024cites this paper
• Gut Pathobiont-Derived Outer Membrane Vesicles Drive Liver Inflammation and Fibrosis in PSC-IBD.

  2024cites this paper
• Hepatic progenitor cell-originated ductular reaction facilitates liver fibrosis through activation of hedgehog signaling

  2024cites this paper
• Gut Microbiota in Primary Sclerosing Cholangitis: From Prognostic Role to Therapeutic Implications

  2024cites this paper
• In Vivo and In Vitro Models to Study Liver Fibrosis: Mechanisms and Limitations

  2023cites this paper
• Prolonged administration of a secretin receptor antagonist inhibits biliary senescence and liver fibrosis in Mdr2−/− mice

  2023cites this paper
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  2023cites this paper
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  2023cites this paper
• Non-steroidal FXR agonist cilofexor improves cholestatic liver injury in the Mdr2-/- mouse model of sclerosing cholangitis

  2023cites this paper
• Oxidative Stress-induced liver damage and remodeling of the liver vasculature.

  2023cites this paper
• Preclinical Models and Promising Pharmacotherapeutic Strategies in Liver Fibrosis: An Update

  2023cites this paper
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  2023cites this paper
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  2022cites this paper
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  2022cites this paper
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  2022cites this paper
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  2022cites this paper
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  2022cites this paper
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  2021cites this paper
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  2021cites this paper
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  2021cites this paper
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  2021cites this paper
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  2020cites this paper
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  2020influential citation
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  2020cites this paper
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  2019cites this paper
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  2019cites this paper
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  2019cites this paper
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  2019cites this paper
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  2018influential citation
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  2018cites this paper
• Fibroinflammatory Liver Injuries as Preneoplastic Condition in Cholangiopathies

  2018cites this paper
• Hydrophilic bile acids prevent liver damage caused by lack of biliary phospholipid in Mdr2−/− mice[S]

  2018cites this paper
• Knockout of secretin receptor reduces biliary damage and liver fibrosis in Mdr2−/− mice by diminishing senescence of cholangiocytes

  2018cites this paper
• Galunisertib modifies the liver fibrotic composition in the Abcb4Ko mouse model

  2018cites this paper
• Interleukin 2 Promotes Hepatic Regulatory T Cell Responses and Protects From Biliary Fibrosis in Murine Sclerosing Cholangitis

  2018cites this paper
• Optimized Mouse Models for Liver Fibrosis.

  2017cites this paper
• Biliary bile acids in hepatobiliary injury - What is the link?

  2017cites this paper
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  2017cites this paper
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  2017cites this paper
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  2017cites this paper
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  2017cites this paper
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  2017cites this paper
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  2016cites this paper
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  2016cites this paper
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  2015cites this paper
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  year unknowncites this paper