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Mouse models of pemphigus: valuable tools to investigate pathomechanisms and novel therapeutic interventions
Autoimmune blistering diseases (AIBD) are paradigms of autoantibody-mediated organ-specific autoimmune disorders that involve skin and/or mucous membranes. Compared to other autoimmune diseases, the pathogenicity of autoantibodies in AIBD is relatively well described. Pemphigus is a potentially...
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| Principais autores: | , , , |
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| Formato: | Artigo |
| Idioma: | inglês |
| Publicado em: |
Philipps-Universität Marburg
2023
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| Assuntos: | |
| Acesso em linha: | Texto Completo em Formato PDF |
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| Resumo: | Autoimmune blistering diseases (AIBD) are paradigms of autoantibody-mediated
organ-specific autoimmune disorders that involve skin and/or mucous
membranes. Compared to other autoimmune diseases, the pathogenicity of
autoantibodies in AIBD is relatively well described. Pemphigus is a potentially
lethal autoantibody driven autoimmune disorder with a strong HLA class II
association. It is mainly characterized by IgG against the desmosomal adhesion
molecules desmoglein 3 (Dsg3) and Dsg1. Several murine pemphigus models
were developed subsequently, each allowing the analysis of a characteristic
feature, such as pathogenic IgG or Dsg3-specific T or B cells. Thus, the models
can be employed to preclinically evaluate potentially novel therapies. We here
thoroughly summarize past and recent efforts in developing and utilizing
pemphigus mouse models for pathomechanistic investigation and
therapeutic interventions. |
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| Descrição do item: | Gefördert durch den Open-Access-Publikationsfonds der UB Marburg. |
| DOI: | 10.3389/fimmu.2023.1169947 |