Abstract
Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic liver disease of unknown etiopathogenesis that mainly affects middle-aged women. Patients show non-suppurative cholangitis with damage and destruction of small- and medium-sized intrahepatic bile ducts. Characteristically, the disease is strongly associated with autoimmune phenomena such as the appearance of serum antimitochondrial autoantibodies (AMA) and portal infiltrates with autoreactive T cells which recognize the inner lipoyl domain of the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Here we review the major characteristics of a series of inducible and genetically modified animal models of PBC and analyze their similarities and differences with PBC features in humans.
Keywords: Break of tolerance against PDC-E2, antimitochondrial autoantibodies, autoimmune cholangitis, autoreactive T cells, cytotoxic CD8+ T cells, defective treg cells, cholestasis, AE2 deficiency, deficient bicarbonate secretion.
Current Pharmaceutical Design
Title:Mouse Models of Primary Biliary Cirrhosis
Volume: 21 Issue: 18
Author(s): Axel R. Concepcion and Juan F. Medina
Affiliation:
Keywords: Break of tolerance against PDC-E2, antimitochondrial autoantibodies, autoimmune cholangitis, autoreactive T cells, cytotoxic CD8+ T cells, defective treg cells, cholestasis, AE2 deficiency, deficient bicarbonate secretion.
Abstract: Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic liver disease of unknown etiopathogenesis that mainly affects middle-aged women. Patients show non-suppurative cholangitis with damage and destruction of small- and medium-sized intrahepatic bile ducts. Characteristically, the disease is strongly associated with autoimmune phenomena such as the appearance of serum antimitochondrial autoantibodies (AMA) and portal infiltrates with autoreactive T cells which recognize the inner lipoyl domain of the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Here we review the major characteristics of a series of inducible and genetically modified animal models of PBC and analyze their similarities and differences with PBC features in humans.
Export Options
About this article
Cite this article as:
Concepcion R. Axel and Medina F. Juan, Mouse Models of Primary Biliary Cirrhosis, Current Pharmaceutical Design 2015; 21 (18) . https://dx.doi.org/10.2174/1381612821666150316121622
DOI https://dx.doi.org/10.2174/1381612821666150316121622 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
Call for Papers in Thematic Issues
Blood-based biomarkers in large-scale screening for neurodegenerative diseases
Disease biomarkers are necessary tools that can be employ in several clinical context of use (COU), ranging from the (early) diagnosis, prognosis, prediction, to monitor of disease state and/or drug efficacy. Regarding neurodegenerative diseases, in particular Alzheimer’s disease (AD), a battery of well-validated biomarkers are available, such as cerebrospinal fluid ...read more
Current Pharmaceutical challenges in the treatment and diagnosis of neurological dysfunctions
Neurological dysfunctions (MND, ALS, MS, PD, AD, HD, ALS, Autism, OCD etc..) present significant challenges in both diagnosis and treatment, often necessitating innovative approaches and therapeutic interventions. This thematic issue aims to explore the current pharmaceutical landscape surrounding neurological disorders, shedding light on the challenges faced by researchers, clinicians, and ...read more
Diabetes mellitus: advances in diagnosis and treatment driving by precision medicine
Diabetes mellitus (DM) is a chronic degenerative metabolic disease with ever increasing prevalence worldwide which is now an epidemic disease affecting 500 million people worldwide. Insufficient insulin secretion from pancreatic β cells unable to maintain blood glucose homeostasis is the main feature of this disease. Multifactorial and complex nature of ...read more
Emerging and re-emerging diseases
Faced with a possible endemic situation of COVID-19, the world has experienced two important phenomena, the emergence of new infectious diseases and/or the resurgence of previously eradicated infectious diseases. Furthermore, the geographic distribution of such diseases has also undergone changes. This context, in turn, may have a strong relationship with ...read more
![Wayfinder Image](/images/wayfinder.jpg)
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Biotechnology in the Development of New Vaccines and Diagnostic Reagents Against Tuberculosis
Current Pharmaceutical Biotechnology The Toxicogenetics of Antirretroviral Therapy: The Evil Inside
Current Medicinal Chemistry Plasma Protein Binding in Drug Discovery and Development
Combinatorial Chemistry & High Throughput Screening Telomere Length Variations in Aging and Age-Related Diseases
Current Aging Science Cannabinoids and Neuro-Inflammation: Regulation of Brain Immune Response
Recent Patents on CNS Drug Discovery (Discontinued) NOX3-Targeted Therapies for Inner Ear Pathologies
Current Pharmaceutical Design Anti-metastatic Treatment in Colorectal Cancer: Targeting Signaling Pathways
Current Pharmaceutical Design Digital Ulcers in Systemic Sclerosis – Frequency, Subtype Distribution and Clinical Outcome
Current Rheumatology Reviews Comparison of Oxidative Stress and Inflammation Induced by Different Intravenous Iron Sucrose Similar Preparations in a Rat Model
Inflammation & Allergy - Drug Targets (Discontinued) Antioxidant Profile of Hyaluronan: Physico-Chemical Features and its Role in Pathologies
Mini-Reviews in Medicinal Chemistry Lipid Rafts and Redox Regulation of Cellular Signaling in Cholesterol Induced Atherosclerosis
Current Cardiology Reviews Adiponectin: Merely a Bystander or the Missing Link to Cardiovascular Disease?
Current Topics in Medicinal Chemistry Novel Therapeutic Targets for the Treatment of Depression
Current Medicinal Chemistry - Central Nervous System Agents Therapeutic Genes for Anti-HIV/AIDS Gene Therapy
Current Pharmaceutical Biotechnology Mexiletine Metabolites: A Review
Current Medicinal Chemistry Near Infrared Optical Technologies to Illuminate the Status of the Neonatal Brain
Current Pediatric Reviews Innate Immunity in Alzheimer’s Disease: A Complex Affair
CNS & Neurological Disorders - Drug Targets β-Catenin/TCF-4 Signaling Regulates Susceptibility of Macrophages and Resistance of Monocytes to HIV-1 Productive Infection
Current HIV Research Mechanisms of Tau Self-Aggregation and Neurotoxicity
Current Alzheimer Research Developments on Drug Delivery Systems for the Treatment of Mycobacterial Infections
Current Topics in Medicinal Chemistry