Autoimmunity and Bullous Pemphigoid
Jaime Brozowski, MS
Baoqi Yang, MD
Ying-Ching Liang, PhD
Cherie Pucheu-Haston, DVM, PhD
Flor Evangelista Montoya
Hemidesmosome and Bullous Pemphigoid. Hemidesmosomes (HDs) are multi-protein complexes connecting the intermediate filament system of basal epithelial cells to proteins of the extracellular matrix of basement membrane. HDs play a critical role in cell-cell matrix adhesion, and any defects of HD functions caused either by gene mutations of HD components or autoantibodies attacking the HD components lead to cell-cell matrix dissociation. Bullous pemphigoid (BP) is an autoimmune inflammatory subepidermal blisterning disease associated with autoantibodies directed against two HD components BP180 (also termed BPAG2 or type XVII collagen) and 230 (BP230 or BPAG1-e).
Dr. Liu's laboratories are credited with the following findings in BP research:
- Developing the animal model of BP by passive transfer of autoantibodies to neonatal mice
- Demonstration that BP autoantibodies recognize HD proteins
- Immunochemical and molecular characterization of the HD protein BP180 (type XVII collagen) as a target antigen recognized by pathogenic BP autoantibodies
Additionally, Dr. Liu's group has developed a humanized BP180 mouse expressing the human NC16a domain of the HD antigen. This unique humanized mouse model is currently in use to dissect the immunological injury triggered by human anti-BP180 autoantibodies.
The research goals of Dr. Liu's laboratory are oriented to the understanding of cell-matrix adhesion proteins of the HD and the structure of the cutaneous basement membrane during normal development and pathogenic conditions. These studies are supported by NIH grants. Our current research projects focus on the following directions:
- Structure and functions of the hemidesmosomal protein BP180 and its associated cell-cell matrix adhesion molecules
- Role of the key innate immune players complement, eosinophils, mast cells, and neutrophils in human skin autoimmune and inflammatory diseases
- Preclinical studies and drug discovery for autoimmune/inflammatory diseases