BP180 as the common autoantigen in blistering diseases with different clinical phenotypes
スポンサーリンク
概要
- 論文の詳細を見る
Bullous pemphigoid antigen 180 (BP180, type XVII collagen) is a transmembrane hemi-desmosomal 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 gestationis (PG), cicatricial pemphigoid (CP), lichen planes pemphigoides (LPP), and linear IgA disease (LAD). The BP180 ectodomain consists of 15 interrupted collagen domains. The largest non-collagenous (NC) 16A domain is located next to the cell membrane. In BP, autoantibodies are directed to a tightly clustered set of epitopes located at the N-terminal 45 amino acids of the NC16A domain. However, some BP sera also react with other por-tions of the BP180 ectodomain or with the intracellular region of this protein. In PG, antibodies appear to exclusively recognize the immunodominant BP180 NC16A region. In CP, autoantibodies are directed to both the NC16A domain and the C-terminus of BP180 that projects into the lamina lucida/ lamina densa interface of the dermal-epidermal junction. In LPP, autoantibodies react with an epitope located at the C-terminus of NC16A, that is not targeted by BP or PG sera. Finally, the epidermal 97 kDa and the keratinoctye-derived 120 kDa autoantigens of LAD (LABD97 and LAD-1, respectively) have recently been identified as portions of the BP180 ectodomain. These observations demonstrate that different clinical phenotypes are associated with an autoinunune response to the same autoantigen yet the immunoglobulin subclass of the autoantibody and the epitope that is recognized may be dif-ferent.
- The Keio Journal of Medicineの論文
- 2002-03-01
著者
関連論文
- Analysis of antigens recognized by autoantibodies in herpes gestationis. Usefulness of immunoblotting using a fusion protein representing an extracellular domain of the 180kD bullous pemphigoid antigen
- Subepidermal blistering disease with autoantibodies against a novel dermal 200-kDa antigen
- Analysis of antigens targeted by circulating IgG and IgA autoantibodies in 50 patients with cicatricial pemphigoid
- From anti-p200 pemphigoid to anti-laminin γ1 pemphigoid
- Epidermolysis bullosa acquisita : What's new?
- Extracellular Cleavage of Bullous Pemphigoid Antigen 180/Type XVII Collagen and Its Involvement in Hemidesmosomal Disassembly
- Cicatricial pemphigoid differs from bullous pemphigoid and pemphigoid gestationis regarding the fine specificity of autoantibodies to the BP180 NC16A domain
- Detection of IL-1α, IL-1β and IL-1 receptor antagonist in blister fluid of bullous pemphigoid
- BP180 as the common autoantigen in blistering diseases with different clinical phenotypes
- Subclass distribution of type VII collagen-specific autoantibodies in patients with inflammatory bowel disease
- Acquired skin disease of hemidesmosomes
- Anti-p200 pemphigoid : A novel autoimmune subepidermal blistering disease
- Development of NC1 and NC2 domains of Type VII collagen ELISA for the diagnosis and analysis of the time course of epidermolysis bullosa acquisita patients