Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa.
case_report · Level V
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Abstract
Immunofluorescence microscopy of epidermodermal junction components in serial cryosections from the perilesional skin of a patient with generalized atrophic benign epidermolysis bullosa (GABEB) showed broken line-like staining of both BPAG2 (180-kDa bullous pemphigoid antigen) and uncein (antigen of 19-DEJ-1 monoclonal antibody), whereas integrin alpha 6 and laminin 5 were continuously expressed along the basement membrane zone. Immunoelectron microscopy revealed a mosaic distribution of the BPAG2/uncein positive and negative cells. BPAG2, a candidate protein of GABEB, probably has a close connection with uncein, and anchoring filament component.
Medical subject headings
- Antigens
- Autoantigens
- Carrier Proteins
- Collagen
- Cytoskeletal Proteins
- Epidermolysis Bullosa
- Nerve Tissue Proteins
- Non-Fibrillar Collagens
- Skin