ArticleThe Journal of investigative dermatology2015
Deletion of the major bullous pemphigoid epitope region of collagen XVII induces blistering, autoimmunization, and itching in mice.
Article in The Journal of investigative dermatology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 38 citations in OpenAlex.
- Complement-Activating Capacity of Autoantibodies Correlates With Disease Activity in Bullous Pemphigoid Patients.Frontiers in immunology · 2018Trial
- Bullous Pemphigoid: A Focused Review on Antigen Epitopes.Experimental dermatology · 2026Review
- IgG autoantibodies in bullous pemphigoid induce a pathogenic MyD88-dependent pro-inflammatory response in keratinocytes.Nature communications · 2025Article
- Pemphigoid disease model systems for clinical translation.Frontiers in immunology · 2025Review
- Anti-NC16A IgA from Patients with Linear IgA Bullous Dermatosis Induce Neutrophil-Dependent Subepidermal Blistering in Mice.The Journal of investigative dermatology · 2024Article
- Integrin alpha 6 homozygous splice-site mutation causes a new form of junctional epidermolysis bullosa in Charolais cattle.Genetics, selection, evolution : GSE · 2023Article
- BP180/Collagen XVII: A Molecular View.International journal of molecular sciences · 2021Review
- Non-Cell-Autonomous Activity of the Hemidesmosomal Protein BP180/Collagen XVII in Granulopoiesis in Humanized NC16A Mice.Journal of immunology (Baltimore, Md. : 1950) · 2020Article
- Collagen XVII Processing and Blistering Skin Diseases.Acta dermato-venereologica · 2020Review
- The dysfunction of BP180/collagen XVII in keratinocytes promotes melanoma progression.Oncogene · 2019Article
- BP180 dysfunction triggers spontaneous skin inflammation in mice.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- The Role of Eosinophils in Bullous Pemphigoid: A Developing Model of Eosinophil Pathogenicity in Mucocutaneous Disease.Frontiers in medicine · 2018Review
- Humoral Epitope Spreading in Autoimmune Bullous Diseases.Frontiers in immunology · 2018Review
- Eosinophils in Autoimmune Diseases.Frontiers in immunology · 2017Review
- BP180 Is Critical in the Autoimmunity of Bullous Pemphigoid.Frontiers in immunology · 2017Review
- Progress toward Treatment and Cure of Epidermolysis Bullosa: Summary of the DEBRA International Research Symposium EB2015.The Journal of investigative dermatology · 2016Review
- Itch, eosinophils, and autoimmunity: a novel murine model of bullous pemphigoid.The Journal of investigative dermatology · 2015Article
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Authors and funding
9 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease with a characteristic of pruritus and blistering. BP patients carry inflammation-triggering autoantibodies against the collagen XVII (ColXVII, also known as BP180) juxtamembraneous extracellular noncollagenous 16A (NC16A) domain involved in ectodomain shedding. Deletion of the corresponding NC14A region in a genetically modified mouse model (ΔNC14A) decreased the amount of ColXVII in skin, but it did not prevent ectodomain shedding. Newborn ΔNC14A mice had no macroscopic phenotypic changes. However, subepidermal microblisters, rudimentary hemidesmosomes, and loose basement membrane zone were observed by microscopy. ΔNC14A mice grow normally, but at around 3 months of age they start to scratch themselves and develop crusted erosions. Furthermore, perilesional eosinophilic infiltrations in the skin, eosinophilia, and elevated serum IgE levels are detected. Despite the removal of the major BP epitope region, ΔNC14A mice developed IgG and IgA autoantibodies with subepidermal reactivity, indicating autoimmunization against a dermo-epidermal junction component. Moreover, IgG autoantibodies recognized a 180-kDa keratinocyte protein, which was sensitive to collagenase digestion. We show here that ΔNC14A mice provide a highly reproducible BP-related mouse model with spontaneous breakage of self-tolerance and development of autoantibodies.
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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.