Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa.
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.
Matsumura
Y
Y
Department of Dermatology, Faculty of Medicine, Kyoto University, Japan.
Horiguchi
Y
Y
Toda
K
K
Fujii
H
H
Kore-Eda
S
S
Tachibana
T
T
Ohta
K
K
Okamoto
H
H
Imamura
S
S
D000328:Adult; D000941:Antigens / Q000032:analysis*; D001324:Autoantigens / Q000032:analysis*; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D004820:Epidermolysis Bullosa / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008297:Male; D008856:Microscopy, Fluorescence; D016253:Microscopy, Immunoelectron; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D012867:Skin / Q000737:chemistry* / Q000648:ultrastructure; D000097805:Collagen Type XVII
D000941:Antigens; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C089136:19-DEJ-1 antigen; C486268:DST protein, human