Abstract
Inflammation of blood vessels (vasculitis) results from many pathological processes and is found in many different diseases. However, in most situations, the pathological processes inducing vasculitis are unknown. The discovery of anti-neutrophil cytoplasmic autoantibodies (ANCAs) in the 1980s opened the door for studies that eventually led to the description of a new previously undescribed disease, ANCA-associated vasculitis (AAV). Unravelling the immunopathogenesis of this new disease resulted largely from the development of animal models. The major breakthroughs were the description of ANCA, its association with small vessel vasculitis and the discovery of its target autoantigens (myeloperoxidase and Proteinase 3). Three major disease syndromes comprise the AAVs, microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA). Recent human studies suggest that proteinase 3 and myeloperoxidase associated vasculitis are two separate but related diseases. The ability to induce murine autoimmunity to myeloperoxidase including ANCA (with the same immune staining patterns as human ANCA) and the capacity of this anti-myeloperoxidase autoimmunity to induce disease with many of the characteristic features of human AAV are well developed. However, the development of animal models of anti-proteinase 3 ANCA and EGPA is much less well developed. Animal models are important in understanding the human disease and in particular in defining potential therapeutic targets and in early stage therapeutic testing of potential drugs. Clearly the relevance of animal models depends on how closely they mimic human diseases. The current status of animal models of vasculitis will be described in detail with reference to these criteria.
Keywords: ANCA, vasculitis, myeloperoxidase, proteinase-3, MPA, GPA, animal models.
Current Pharmaceutical Design
Title:Mouse Models of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Volume: 21 Issue: 18
Author(s): Poh-Yi Gan, Joshua D. Ooi, A. Richard Kitching and Stephen R. Holdsworth
Affiliation:
Keywords: ANCA, vasculitis, myeloperoxidase, proteinase-3, MPA, GPA, animal models.
Abstract: Inflammation of blood vessels (vasculitis) results from many pathological processes and is found in many different diseases. However, in most situations, the pathological processes inducing vasculitis are unknown. The discovery of anti-neutrophil cytoplasmic autoantibodies (ANCAs) in the 1980s opened the door for studies that eventually led to the description of a new previously undescribed disease, ANCA-associated vasculitis (AAV). Unravelling the immunopathogenesis of this new disease resulted largely from the development of animal models. The major breakthroughs were the description of ANCA, its association with small vessel vasculitis and the discovery of its target autoantigens (myeloperoxidase and Proteinase 3). Three major disease syndromes comprise the AAVs, microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA). Recent human studies suggest that proteinase 3 and myeloperoxidase associated vasculitis are two separate but related diseases. The ability to induce murine autoimmunity to myeloperoxidase including ANCA (with the same immune staining patterns as human ANCA) and the capacity of this anti-myeloperoxidase autoimmunity to induce disease with many of the characteristic features of human AAV are well developed. However, the development of animal models of anti-proteinase 3 ANCA and EGPA is much less well developed. Animal models are important in understanding the human disease and in particular in defining potential therapeutic targets and in early stage therapeutic testing of potential drugs. Clearly the relevance of animal models depends on how closely they mimic human diseases. The current status of animal models of vasculitis will be described in detail with reference to these criteria.
Export Options
About this article
Cite this article as:
Gan Poh-Yi, Ooi D. Joshua, Kitching Richard A. and Holdsworth R. Stephen, Mouse Models of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Current Pharmaceutical Design 2015; 21 (18) . https://dx.doi.org/10.2174/1381612821666150316121029
DOI https://dx.doi.org/10.2174/1381612821666150316121029 |
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
-
Gene Therapy for Ocular Neovascularization
Current Gene Therapy Polyphenolic Compounds and Gut Microbiome in Cardiovascular Diseases
Current Pharmaceutical Biotechnology Extracellular Matrix on the Phenotypic Switching of Vascular Smooth Muscle Cells
Current Angiogenesis (Discontinued) Pharmacogenetics and Statin Treatment: Reality or Theory?
Current Vascular Pharmacology Patent Selections :
Recent Patents on Cardiovascular Drug Discovery Potassium Channels and Uterine Vascular Adaptation to Pregnancy and Chronic Hypoxia
Current Vascular Pharmacology Anti-Inflammatory and Anti-Allergy Drugs in Rhinosinusitis
Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry The Role of Anti-Inflammatory Drugs in Respiratory Diseases - Pirfenidone, Penicillamine, Chloroquine and Chlorambucil
Current Respiratory Medicine Reviews Olanzapine-Induced Reversible Pellagroid Skin Lesion
Current Drug Safety Targeting Chemoattractant Receptors in Allergic Inflammation
Current Drug Targets - Inflammation & Allergy Transcription Factors in Autoimmune Diseases
Current Pharmaceutical Design Epigenetic control of cardiovascular health by nutritional polyphenols involves multiple chromatin-modifying writer-reader-eraser proteins
Current Topics in Medicinal Chemistry Biologic Agents in the Treatment of Psoriasis
Recent Patents on Inflammation & Allergy Drug Discovery Selenium and Autoimmune Diseases: A Review Article
Current Rheumatology Reviews Cardiovascular Pharmacogenetics of Antihypertensive and Lipid- Lowering Therapies
Current Molecular Medicine Renin Angiotensin System (RAS) and Immune System Profile in Specific Subgroups with COVID-19
Current Medicinal Chemistry A Systematic Approach to Hypertensive Urgencies and Emergencies
Current Hypertension Reviews Modulation of Angiotensin II Effects, A Potential Novel Approach to Inflammatory and Immune Diseases
Current Medicinal Chemistry - Anti-Inflammatory & Anti-Allergy Agents Alpha-1 Antitrypsin Deficiency: Current Perspective from Genetics to Diagnosis and Therapeutic Approaches
Current Medicinal Chemistry Effects on Cytokines and Histology by Treatment with the Ace Inhibitor Captopril and the Antioxidant Retinoic Acid in the Monocrotaline Model of Experimentally Induced Lung Fibrosis
Current Pharmaceutical Design