Autoimmune blistering skin diseases
BP180, a protein expressed by keratinocytes in the basal layer of the epidermis, plays a prominent role in several blistering skin diseases – both acquired and inherited. Our laboratory has shown that autoantibodies against BP180 play a critical role in the pathogenesis of two such diseases, bullous pemphigoid (a disease of the elderly) and pemphigoid gestationis (a pregnancy-associated disease). Key factors in the multi-step pathogenic mechanisms were elucidated through our use of various animal models. Our recent work in collaboration with Dr. Janet Fairley (Head of Dermatology, U. Iowa) represents the first time a pathogenic role has been ascribed to a specific IgE class autoantibody in an autoimmune disease.
Insights into the structural and functional properties of BP180 were gained through our immunological analyses, as well as our biochemical studies of BP180 mutations that are known to be responsible for the inherited blistering disease, non-Herlitz junctional epidermolysis bullosa. Future directions that our lab will be taking include identifying BP180 interactor molecules, further probing the signaling properties of BP180 (both autoantibody-mediated and ligand-mediated) and continuing our investigations into other autoimmune blistering skin diseases.