Abstract
Autoimmune bullous dermatoses (AIBD), such as pemphigus, bullous pemphigoid or epidermolysis bullosa acquisita, are prototypical organ-specific autoimmune diseases. Clinically they are characterized by widespread mucocutaneous blistering, which is often difficult to treat. Patients with AIBD suffer from a significant morbidity and an increased mortality. In AIBD blistering is caused by autoantibodies targeting structural proteins of the skin. During the past decades animal models of AIBD have been developed. These animal models have greatly contributed to our current understanding of AIBD pathogenesis. Most of these insights, however, still await their translation into clinical use. Recently, AIBD animal models have been used to test the efficacy of known and novel drugs. Hence, these models are now not only employed to unravel the pathogenesis of AIBD, but also to assess therapeutic approaches to address the so far unmet high medical need for new treatments. We here review animal model of AIBD: In addition to spontaneously arising AIBD in animals, AIBD can be induced, mostly in mice, by (i) transfer of (auto)-antibodies, (ii) transfer of (auto)-antigen specific lymphocytes, (iii) immunization or (iv) by genetic modifications leading to spontaneous blistering. In combined use, these models allow dissecting all aspects of AIBD pathogenesis, i.e. loss of tolerance, autoantibody production and blistering. Overall we aim to foster a broader use of AIBD animal models, especially in translational biomedical research, to deepen our understanding of AIBD pathogenesis and to develop novel treatments for patients.
Keywords: Autoimmunity, autoimmune bullous dermatoses, skin, animal model, pemphigus, pemphigoid, epidermolysis bullosa acquisita.
Current Pharmaceutical Design
Title:Animal Models to Investigate Pathomechanisms and Evaluate Novel Treatments for Autoimmune Bullous Dermatoses
Volume: 21 Issue: 18
Author(s): Hiroaki Iwata, Katja Bieber, Misa Hirose and Ralf J. Ludwig
Affiliation:
Keywords: Autoimmunity, autoimmune bullous dermatoses, skin, animal model, pemphigus, pemphigoid, epidermolysis bullosa acquisita.
Abstract: Autoimmune bullous dermatoses (AIBD), such as pemphigus, bullous pemphigoid or epidermolysis bullosa acquisita, are prototypical organ-specific autoimmune diseases. Clinically they are characterized by widespread mucocutaneous blistering, which is often difficult to treat. Patients with AIBD suffer from a significant morbidity and an increased mortality. In AIBD blistering is caused by autoantibodies targeting structural proteins of the skin. During the past decades animal models of AIBD have been developed. These animal models have greatly contributed to our current understanding of AIBD pathogenesis. Most of these insights, however, still await their translation into clinical use. Recently, AIBD animal models have been used to test the efficacy of known and novel drugs. Hence, these models are now not only employed to unravel the pathogenesis of AIBD, but also to assess therapeutic approaches to address the so far unmet high medical need for new treatments. We here review animal model of AIBD: In addition to spontaneously arising AIBD in animals, AIBD can be induced, mostly in mice, by (i) transfer of (auto)-antibodies, (ii) transfer of (auto)-antigen specific lymphocytes, (iii) immunization or (iv) by genetic modifications leading to spontaneous blistering. In combined use, these models allow dissecting all aspects of AIBD pathogenesis, i.e. loss of tolerance, autoantibody production and blistering. Overall we aim to foster a broader use of AIBD animal models, especially in translational biomedical research, to deepen our understanding of AIBD pathogenesis and to develop novel treatments for patients.
Export Options
About this article
Cite this article as:
Iwata Hiroaki, Bieber Katja, Hirose Misa and Ludwig J. Ralf, Animal Models to Investigate Pathomechanisms and Evaluate Novel Treatments for Autoimmune Bullous Dermatoses, Current Pharmaceutical Design 2015; 21 (18) . https://dx.doi.org/10.2174/1381612821666150316122502
DOI https://dx.doi.org/10.2174/1381612821666150316122502 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
Call for Papers in Thematic Issues
"Tuberculosis Prevention, Diagnosis and Drug Discovery"
The Nobel Prize-winning discoveries of Mycobacterium tuberculosis and streptomycin have enabled an appropriate diagnosis and an effective treatment of tuberculosis (TB). Since then, many newer diagnosis methods and drugs have been saving millions of lives. Despite advances in the past, TB is still a leading cause of infectious disease mortality ...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
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
Melanoma and Non-Melanoma Skin Cancer Treatment: Standard of Care and Recent Advances
In this thematic issue, we aim to provide a standard of care of the diagnosis and treatment of melanoma and non-melanoma skin cancer. The editor will invite authors from different countries who will write review articles of melanoma and non-melanoma skin cancers. The Diagnosis, Staging, Surgical Treatment, Non-Surgical Treatment all ...read more
- 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
-
Protein Engineering Studies for C-C Chemokine Receptor Type 2 (CCR2)
Current Enzyme Inhibition Biologics in Inflammatory Immune-mediated Systemic Diseases
Current Pharmaceutical Biotechnology Autoimmunity-Inducing Metals (Hg, Au and Ag) Modulate Mast Cell Signaling, Function and Survival
Current Pharmaceutical Design Review of Recent Clinical Developments and Patents for the Treatment of Autoimmune and Inflammatory Diseases by Mesenchymal Stromal Cells
Recent Patents on Regenerative Medicine Natural Cures for Type 1 Diabetes: A Review of Phytochemicals, Biological Actions, and Clinical Potential
Current Medicinal Chemistry Anti-Interleukin-6 Receptor Antibody Treatment in Inflammatory Autoimmune Diseases
Reviews on Recent Clinical Trials Tuning Immune Suppression in Systemic Autoimmunity with Self-Derived Peptides
Inflammation & Allergy - Drug Targets (Discontinued) Control of Autoimmune Diseases by the B7-CD28 Family Molecules
Current Pharmaceutical Design Antiphospholipid Antibody-Mediated Thrombotic Mechanisms in Antiphospholipid Syndrome: Towards Pathophysiology-Based Treatment
Current Pharmaceutical Design Phospholipid/Bile Salt Based Novel Mixed Nanomicelles of Methotrexate Co-encapsulated with Sesamol: Preparation, Characterization, and Evaluation of Antiradical Effects In Vitro
Current Nanomedicine Mechanisms Leading to Autoantibody Production: Link Between Inflammation and Autoimmunity
Current Drug Targets - Inflammation & Allergy HSP60 is a Ubiquitous Player in the Physiological and Pathogenic Interactions between the Chaperoning and the Immune Systems
Current Immunology Reviews (Discontinued) Micro- and Nano-particulate Strategies for Antigen Specific Immune Tolerance to Treat Autoimmune Diseases
Pharmaceutical Nanotechnology B Lymphocytes, Potent Antigen Presenting Cells for Preferential Expansion of Allo-Reactive FoxP3+ CD4 Regulatory T Cells
Recent Patents on Endocrine, Metabolic & Immune Drug Discovery Role of Regulatory T Cells in Allergy: Implications for Therapeutic Strategy
Inflammation & Allergy - Drug Targets (Discontinued) The Janus Face of CD4+CD25+ Regulatory T Cells in Cancer and Autoimmunity
Current Medicinal Chemistry Stem Cell-Based Immunomodulation in Type 1 Diabetes: Beyond the Regenerative Approach
Current Pharmaceutical Design Biochemical Markers of Autoimmune Diseases of the Nervous System
Current Pharmaceutical Design Models of Antigen Receptor Activation in the Design of Vaccines
Current Pharmaceutical Design Immunotherapy for Multiple Sclerosis: Basic Insights for New Clinical Strategies
Current Neurovascular Research