antigen defect in Pemphigus Vulgaris -
Antigen defect in Pemphigus Vulgaris is desmoglein 1 & 3. When adsorbed with extracellular domains of both Dsg3 and Dsg1, these sera no longer stained epidermis, demonstrating that most. if not all, of their cell surface reactivity can be attributed to antibodies against the extracellular domains of these desmogleins. Adsorption with only the Dsg1 extracellular domain left antibodies that stained only the basal and immediate suprabasal layers of the epidermis and immunoprecipitated only Dsg3, not Dsg1, from extracts of cultured cells synthesizing these molecules. In contrast, adsorption with only the Dsg3 extracellular domain Left antibodies that stained only the more superficial epidermis and immunoprecipitated only Dsg1. These data localize Dsg3 exactly to the area in the epidermis where Blisters occur in pemphigus vulgaris.
Desmoglein-1
Desmoglein-3
Desmocolin-3
Desmocollin-2
Vestibulobullous disorders