Abstract: Pemphigus vulgaris (PV) is an autoimmune disease with a possible fatality of the skin and mucosae which is induced by an antibody against desmoglein 3 (Dsg3). Persistent production of anti-Dsg3 IgG can be induced by adoptively transferring spleen cells of a DSG3?/? mouse immunized with rDsg3 into an RAG2?/? immunodeficient mouse expressing Dsg3 protein. This IgG in the blood binds to the Dsg3 protein in vivo, induces the breakage of intercellular adhesion of keratinocytes and thus brings about the phenotype of pemphigus vulgaris involving the formation of blisters in the oral mucosa and the disappearance of resting hair. These effects are sustained over 6 months. By using this method, active disease model animals relating to various autoimmune diseases can be constructed.

Abstract: The present invention provides a monoclonal antibody having a pathogenic activity that can induce pemphigus lesion, a peptide specifically recognized by the monoclonal antibody and useful as a therapeutic drug for pemphigus autoimmune disease, etc. As anti-mouse Dsg3 antibody-producing cells are present in the splenocytes of pemphigus vulgaris mouse model constructed by using autoantigen knockout mouse, cell fusion was conducted with the splenocytes of said mouse model and mouse myeloma cells using polyethyleneglycol, hybridomas were constructed, and monoclonal antibodies against Dsg3 were constructed. Among them, a monoclonal antibody having a pathological activity that can induce pemphigus lesions was screened, the base sequence and amino acid sequence in its variable region (heavy chain, light chain) was determined, and the specific epitope part was identified.

Abstract: The present invention concerns developing a remedy for treating pemphigus by administering a CD40L antagonist to patients with pemphigus such as pemphigus vulgaris or pemphigus foliaceus, and a preventive agent for preventing the development of pemphigus by preventively administering a CD40L antagonist. Antagonists such as an anti-CD40L antibody that inhibit the interaction between a CD40L mediating the contact-dependent helper effector function on the T cell surface and a CD40 receptor on the antigen-presenting cell surface, were effective as a remedy and a preventive agent for pemphigus.

Abstract: Pemphigus vulgaris (PV) is an autoimmune disease with a possible fatality of the skin and mucosae which is induced by an antibody against desmoglein 3 (Dsg3). Persistent production of anti-Dsg3 IgG can be induced by adoptively transferring spleen cells of a DSG3?/? mouse immunized with rDsg3 into an RAG2?/? immunodeficient mouse expressing Dsg3 protein. This IgG in the blood binds to the Dsg3 protein in vivo, induces the breakage of intercellular adhesion of keratinocytes and thus brings about the phenotype of pemphigus vulgaris involving the formation of blisters in the oral mucosa and the disappearance of resting hair. These effects are sustained over 6 months. By using this method, active disease model animals relating to various autoimmune diseases can be constructed.

Abstract: Pemphigus vulgaris (PV) is an autoimmune disease with a possible fatality of the skin and mucosae which is induced by an antibody against desmoglein 3 (Dsg3). Persistent production of anti-Dsg3 IgG can be induced by adoptively transferring spleen cells of a DSG3?/? mouse immunized with rDsg3 into an RAG2?/? immunodeficient mouse expressing Dsg3 protein. This IgG in the blood binds to the Dsg3 protein in vivo, induces the breakage of intercellular adhesion of keratinocytes and thus brings about the phenotype of pemphigus vulgaris involving the formation of blisters in the oral mucosa and the disappearance of resting hair. These effects are sustained over 6 months. By using this method, active disease model animals relating to various autoimmune diseases can be constructed.

Abstract: The present invention provides a remedy to be used in gene therapy that can suppress immune response to a transgene product in gene therapy of genetic diseases such as recessive genetic diseases, or a method for treating genetic diseases such as recessive genetic diseases with the use of the remedy to be used in gene therapy. A remedy to be used in gene therapy is prepared with an immunosuppressive agent and a gene responsible for recessive genetic diseases such as recessive genetic epidermolysis bullosa hereditaria dystrophica, junctional epidermolysis bullosa hereditaria, hemidesmosome. epidermolysis bullosa hereditaria, ichthyosis congenita. As for the above-mentioned immunosuppressive agent, cyclosporin and anti-CD40L antibody can be exemplified. Moreover, the gene responsible for genetic diseases above mentioned can be used in a form of viral vector, and a form of naked DNA.

Abstract: The present invention provides a monoclonal antibody having a pathogenic activity that can induce pemphigus lesion, a peptide specifically recognized by the monoclonal antibody and useful as a therapeutic drug for pemphigus autoimmune disease, etc. As anti-mouse Dsg3 antibody-producing cells are present in the splenocytes of pemphigus vulgaris mouse model constructed by using autoantigen knockout mouse, cell fusion was conducted with the splenocytes of said mouse model and mouse myeloma cells using polyethyleneglycol, hybridomas were constructed, and monoclonal antibodies against Dsg3 were constructed. Among them, a monoclonal antibody having a pathological activity that can induce pemphigus lesions was screened, the base sequence and amino acid sequence in its variable region (heavy chain, light chain) was determined, and the specific epitope part was identified.