Autoantigen-Humanized Mouse Models of Bullous Pemphigoid.

Area of Science:

• Immunodermatology
• Autoimmune Blistering Diseases
• Preclinical Research Models

Background:

• Bullous pemphigoid (BP) is an autoimmune blistering disease primarily affecting the elderly, characterized by subepidermal blisters and inflammation.
• Current treatments like oral corticosteroids have significant limitations and adverse effects in elderly patients, necessitating novel therapeutic strategies.
• Existing mouse models for BP often lack consistency in disease induction, hindering reliable therapeutic investigations.

Purpose of the Study:

• To describe detailed methodologies for establishing reliable bullous pemphigoid mouse models.
• To present protocols for both active and passive IgG transfer mouse models using COL17-humanized mice.
• To facilitate preclinical therapeutic studies and the elucidation of BP pathophysiology.

Main Methods:

• Development of an active bullous pemphigoid (BP) mouse model using COL17-humanized mice.
• Implementation of a neonatal passive IgG transfer mouse model with COL17-specific antibodies.
• Detailed description of materials and methods for both established mouse models.

Main Results:

• The described active BP mouse model reliably recapitulates key features of human bullous pemphigoid.
• The neonatal passive IgG transfer model offers a valuable system for antigen-specific therapy development.
• These models provide a foundation for investigating bullous pemphigoid pathophysiology and preclinical therapeutic testing.

Conclusions:

• Reliable mouse models, particularly autoantigen-humanized systems, are essential for advancing bullous pemphigoid research.
• The active and passive transfer models presented are valuable tools for preclinical therapeutic studies and understanding disease mechanisms.
• Further research utilizing these models can accelerate the development of targeted therapies for bullous pemphigoid.