Anti-BP180, BP230, Desmoglein-1, Desmoglein 3
Cell-Based Assay (CBA)
Qualitative Analysis: Neg /Low Positive/Med Positive/High Positive
Mitogen’s Bullous Autoimmune Skin Disease Profile is an autoimmune diagnostic test that supports the diagnosis of autoimmune cutaneous bullous diseases such as bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF). BP180 (also known as collagen XVII or bullous pemphigoid antigen 2) is a transmembrane protein that is responsible for epidermal cell / tissue adhesion. BP230 (also know as bullous pemphigoid antigen 1 or BPAG1) is a cytoplasmic protein found in keratinocytes (epidermal cells). It interacts with BP180 and integrins at the cell membrane and it is responsible for the attachment of keratin filaments to the hemidesmosomal plaque and hemidesmosomes in general. Hemidesmosomes are cellular structures of keratinocytes that attach these epidermal cells to the extracellular matrix. Autoantibodies to BP180 and BP230 are prevalent in bullous pemphigoid
Desmoglein-1 and Desmoglien 3 are calcium-binding transmembrane glycoproteins that is prevalent throughout the skin epidermis. Desmoglein-1 is primarily expressed in the superficial upper layers of the skin epidermis whereas Desmoglein-3 is expressed in the basal lower layers of the epidermis. These proteins are part of desmosomes – a cellular junction complex that adhere epidermal cells. Autoantibodies to Desmoglein-1 is prevalent in pemphigus foliaceus whereas autoantibodies to Desmoglein-3 is prevalent in pemphigus vulgaris.
Reference: S. Egami, J. Yamagami, and M. Amagai. Autoimmune bullous skin diseases, pemphigus and pemphigoid. J Allergy Clin