<> "The repository administrator has not yet configured an RDF license."^^ . <> . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice"^^ . "Previous studies have shown that regulatory T cell-deficient scurfy mice develop several autoimmune diseases including autoimmune blistering disease (AIBD), associated with the production of high titers of anti-basement membrane antibodies.\r\nTo approach the cellular mechanisms of autoimmunity, this project demonstrated that scurfy mice are characterized by elevated levels of several B cell subpopulations, including germinal center B cells, memory B cells, and plasma cells. Focusing on the site of AIBD manifestation, scurfy mice showed significantly higher numbers of B cells in the skin. To further specify the role of B cells in the pathogenesis of AIBD, scurfy mice exhibited antigen-specific B cells against human relevant AIBD-related proteins present in the skin. Among others, Collagen type VII (Col7), the immunodominant antigen of the human disease Epidermolysis bullosa acquisita (EBA), was identified. \r\nTo study the immune response to this antigen in more detail, the scurfy-derived monoclonal antibody H510 was used which had previously been shown to be pathogenic in vivo as injection of H510 into wildtype neonatal mice resulted in subepidermal blisters. In the present project the murine von-Willebrand-Factor-A-like domain 2 of Col7 was identified as the target epitope of H510. Focusing on the pathogenic mechanism, injection into Fcγ-receptor knock-out mice could not abrogate the blister-inducing capacity of this anti-Col7 antibody, suggesting that H510 acts independently of cell-induced inflammation.\r\nIn conclusion, this project demonstrates the relevance of antigen-specific B cells for AIBD induction in scurfy mice, highlighting their potential role as a therapeutic target in human AIBD. Furthermore, the identification of the pathogenic anti-Col7 antibody H510 provides a new EBA mouse model that might become an important tool to further study this rare human AIBD and allow for potential development of novel therapeutic targets."^^ . "2025" . . . . . . . "Elisabeth Marie"^^ . "Vicari"^^ . "Elisabeth Marie Vicari"^^ . . . . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (PDF)"^^ . . . "Elisabeth Vicari_Doktorthesis.pdf"^^ . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (Other)"^^ . . . . . . "lightbox.jpg"^^ . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (Other)"^^ . . . . . . "preview.jpg"^^ . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (Other)"^^ . . . . . . "medium.jpg"^^ . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (Other)"^^ . . . . . . "small.jpg"^^ . . . "Contribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice (Other)"^^ . . . . . . "indexcodes.txt"^^ . . "HTML Summary of #35830 \n\nContribution of B cells to autoimmune blistering diseases in regulatory T cell-deficient scurfy mice\n\n" . "text/html" . . . "570 Biowissenschaften, Biologie"@de . "570 Life sciences"@en . . . "610 Medizin"@de . "610 Medical sciences Medicine"@en . .