This study demonstrates that human IgG targeting the membrane-proximal EC5 domain of desmoglein 3 (DSG3) disrupts keratinocyte adhesion and induces hallmark blistering of pemphigus vulgaris. In vivo experiments using a humanized DSG3 Knockin mouse model confirm that EC5-specific antibodies are pathogenic and sufficient to induce disease features, supporting the role of epitope specificity in autoimmunity.
EC5-specific anti-DSG3 IgG causes loss of keratinocyte adhesion and blistering in humanized mice, confirming the direct pathogenicity of this epitope in pemphigus vulgaris.
Humanized DSG3 Knockin mouse model developed by genOway, with human DSG3 cDNA replacing the murine gene, enabling interaction with human PV autoantibodies in vivo.
Pemphigus vulgaris, Autoimmune skin disease, Epitope specificity, Keratinocyte adhesion, Human antibody interaction
Humanized Knockin model, DSG3 gene replacement, Autoimmune disease modeling, Antibody transfer assay
From model design to experimental results
Tailor-made solutions adapted to scientific questions
Comprehensive dataset package
Generated with biopharma partners and in-house
Scientific follow-up and advice along the project
Collaborative approach for problem solving and development of innovative models
Breeding facilities in US and Europe
Certified health status from professional breeders
