泛发性萎缩性良性大疱性表皮松解症中uncenin和180 kDa大疱性类天疱疮抗原的镶嵌性表达

Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa.

作者信息

Matsumura Y, Horiguchi Y, Toda K, Fujii H, Kore-Eda S, Tachibana T, Ohta K, Okamoto H, Imamura S

机构信息

Department of Dermatology, Faculty of Medicine, Kyoto University, Japan.

出版信息

Br J Dermatol. 1997 May;136(5):757-61.

PMID:9205513

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.

摘要

对一名泛发性萎缩性良性大疱性表皮松解症（GABEB）患者病损周围皮肤连续冰冻切片进行表皮真皮连接成分的免疫荧光显微镜检查显示，BPAG2（180 kDa大疱性类天疱疮抗原）和uncein（19-DEJ-1单克隆抗体的抗原）均呈折线状染色，而整合素α6和层粘连蛋白5沿基底膜带持续表达。免疫电子显微镜检查显示BPAG2/uncein阳性和阴性细胞呈镶嵌分布。BPAG2作为GABEB的候选蛋白，可能与uncein及锚定丝成分密切相关。