ReviewClinical reviews in allergy & immunology2015
Animal models in primary biliary cirrhosis and primary sclerosing cholangitis.
Review in Clinical reviews in allergy & immunology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
26 citing papers in PubMed, 2 syntheses or guidelines pooled it, 53 citations in OpenAlex.
- Pooled it
- Clinical and Immunological Features, Genetic Variants, and Outcomes of Patients with CD40 Deficiency.Journal of clinical immunology · 2023Pooled it
- Disruption of Bile Acid Metabolism in the Gut-Liver Axis Predisposes Mice to Inflammatory Bowel Disease.MedComm · 2025Article
- Article
- Liver GPBAR1 Associates With Immune Dysfunction in Primary Sclerosing Cholangitis and Its Activation Attenuates Cholestasis in Abcb4-/- Mice.Liver international : official journal of the International Association for the Study of the Liver · 2025Article
- Human vascularized bile duct-on-a chip: a multi-cellular micro-physiological system for studying cholestatic liver disease.Biofabrication · 2023Article
- NOTCH signalling - a core regulator of bile duct disease?Disease models & mechanisms · 2023Review
- Metabolomic Analysis Reveals the Therapeutic Effects of MBT1805, a Novel Pan-Peroxisome Proliferator-Activated Receptor Agonist, on α-Naphthylisothiocyanate-Induced Cholestasis in Mice.Frontiers in pharmacology · 2021Article
- Mammalian Target of Rapamycin Complex 2 Signaling Is Required for Liver Regeneration in a Cholestatic Liver Injury Murine Model.The American journal of pathology · 2020Article
- Animal Models of Autoimmune Liver Diseases: a Comprehensive Review.Clinical reviews in allergy & immunology · 2020Review
- Preclinical insights into cholangiopathies: disease modeling and emerging therapeutic targets.Expert opinion on therapeutic targets · 2019Review
- Fibroinflammatory Liver Injuries as Preneoplastic Condition in Cholangiopathies.International journal of molecular sciences · 2018Review
- Cholangiopathies - Towards a molecular understanding.EBioMedicine · 2018Review
- Proteomic analysis reveals distinctive protein profiles involved in CD8Cellular & molecular immunology · 2018Article
- Animal models of biliary injury and altered bile acid metabolism.Biochimica et biophysica acta. Molecular basis of disease · 2018Review
- Article
- Effects of human amnion-derived mesenchymal stem cells and conditioned medium in rats with sclerosing cholangitis.American journal of translational research · 2018Article
- Inhibition of JNK signalling mediates PPARα-dependent protection against intrahepatic cholestasis by fenofibrate.British journal of pharmacology · 2017Article
- Trichloroethylene Exposure Reduces Liver Injury in a Mouse Model of Primary Biliary Cholangitis.Toxicological sciences : an official journal of the Society of Toxicology · 2017Article
- The gut microbiota contributes to a mouse model of spontaneous bile duct inflammation.Journal of hepatology · 2017Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are immune-mediated cholangiopathies with enigmatic etiology and pathogenesis. They have distinct clinical, laboratory, immunological, and histomorphological characteristics. Well-characterized animal models for PBC and PSC are utterly needed to develop novel pathogenetic concepts and to study innovative treatment strategies. The aim of the current paper is to outline the characteristics of ideal PBC and PSC animal models and to contrast this with a real-life up-to-date overview of currently available mouse models. Although some of this models show several individual characteristics of PBC and PSC, it is obvious that all of them have substantial and important limitations. Nevertheless, some may be beneficial to study certain pathophysiological aspects. Potential cholangiopathy animal models should be systematically investigated in regard to elevated serum alkaline phosphatase, bilirubin, and bile acid levels; immunological abnormalities; and longitudinal studies in regard to their liver phenotype. We herein propose a common systematic workup for potential models based on the fact that there are some intriguing disease combinations in specific genetically modified mice and recommend a stepwise process in regard to model characterization with methodical harvesting and screening of numerous organs for potential concomitant diseases. Due to the complex nature of both cholangiopathies, it seems to be very likely that no single perfect PBC or PSC model will ever be generated. The models outlined herein will certainly help to clarify specific pathogenetic aspects and even more important may turn out to be suitable to test potential drugs for treatment.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.