Bullous Pemphigoid and Cicatricial Pemphigoid Autoantibodies React with Ultrastructurally Separable Epitopes on the BP180 Ectodomain: Evidence that BP180 Spans the Lamina Lucida
نویسندگان
چکیده
منابع مشابه
BP180 as the common autoantigen in blistering diseases with different clinical phenotypes.
Bullous pemphigoid antigen 180 (BP180, type XVII collagen) is a transmembrane hemidesmosomal glycoprotein of basal keratinocytes that spans the lamina lucida of the dermal-epidermal junction. Five autoimmune subepidermal blistering diseases are associated with an immune response to BP180, including bullous pemphigoid (BP), pemphigoid gestations (PG), cicatricial pemphigoid (CP), lichen planus p...
متن کاملBullous pemphigoid of childhood: autoantibodies target the same epitopes within the NC16A domain of BP180 as autoantibodies in bullous pemphigoid of adulthood.
BACKGROUND Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease of the elderly that rarely occurs in children. Most adult BP serum samples react with epitopes within the NC16A domain of BP180, a glycoprotein of the cutaneous basement membrane zone. OBJECTIVES To characterize the autoimmune response in childhood BP using recombinant forms of BP180 and to determine the subcl...
متن کاملBP230- and BP180-specific auto-antibodies in bullous pemphigoid.
Bullous pemphigoid is a subepidermal blistering disease associated with auto-antibodies (auto-ab) to BP180 and BP230. We developed ELISAs utilizing baculovirus-encoded recombinant proteins of BP230 and BP180 and studied their diagnostic and prognostic values by assessing the profile of the auto-ab response in 127 patients with BP. 39 patients had focal involvement, whereas 88 had generalized di...
متن کاملBP180 Is Critical in the Autoimmunity of Bullous Pemphigoid
Bullous pemphigoid (BP) is by far the most common autoimmune blistering dermatosis that mainly occurs in the elderly. The BP180 is a transmembrane glycoprotein, which is highly immunodominant in BP. The structure and location of BP180 indicate that it is a significant autoantigen and plays a key role in blister formation. Autoantibodies from BP patients react with BP180, which leads to its degr...
متن کاملSerum Levels of Autoantibodies to BP180 Correlate With Disease Activity in Patients With Bullous Pemphigoid
Main Outcome Measures: Disease activity, serum levels of autoantibodies to BP180, and titers of anti– basement membrane zone autoantibodies were assayed before initiation of treatment and 4 and 8 weeks later. Reactivity to BP180 was analyzed by enzyme-linked immunosorbent assay using a recombinant form of BP180 NC16A. Titers of anti–basement membrane zone autoantibodies were assayed by indirect...
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ژورنال
عنوان ژورنال: Journal of Investigative Dermatology
سال: 1997
ISSN: 0022-202X
DOI: 10.1111/1523-1747.ep12292701