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首页> 外文期刊>Archives of dermatological research. >BP180/type XVII collagen: its role in acquired and inherited disorders or the dermal-epidermal junction.
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BP180/type XVII collagen: its role in acquired and inherited disorders or the dermal-epidermal junction.

机译：BP180 / XVII型胶原蛋白：其在获得性和遗传性疾病或真皮-表皮交界处的作用。

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摘要

BP180 is a member of the collagen protein family and is also referred to as type XVII collagen or BP antigen 2. It is a transmembrane protein constituent of the dermal-epidermal anchoring complex. The long-held hypothesis that BP180 functions as a cell-matrix adhesion molecule has been supported by recent investigations of human disorders of the dermal-epidermal junction in which BP180 is either genetically defective or targeted by the immune system. In generalized atrophic benign epidermolysis bullosa, mutations of BP180 result in an inherited subepidermal blistering disease. In bullous pemphigoid, herpes/pemphigoid gestationis, cicatricial pemphigoid, lichen planus pemphigoides and linear IgA disease, autoantibodies are directed to different epitopes on the BP180 ectodomain. Recent molecular investigations have provided new insights, not only into the mechanism of autoantibody-mediated subepidermal blistering, but also into the biochemical structure and cell biological functions of BP180 and other components of the dermal-epidermal anchoring complex. These findings have suggested new directions for the development of diagnostic and therapeutic tools for these autoimmune and genetic diseases.
机译：BP180是胶原蛋白家族的成员，也称为XVII型胶原蛋白或BP抗原2。它是真皮-表皮锚固复合物的跨膜蛋白成分。长期以来一直存在的关于BP180充当细胞-基质粘附分子的假说已经得到了对人类皮肤-表皮交界处疾病的最新研究的支持，其中BP180是遗传缺陷或被免疫系统靶向。在广义萎缩性良性表皮松解性大疱中，BP180突变导致遗传性表皮下水疱病。在大疱性天疱疮，疱疹/天疱疮妊娠，瘢痕性天疱疮，扁平苔藓天疱疮和线性IgA疾病中，自身抗体针对BP180胞外域上的不同表位。最近的分子研究不仅为自身抗体介导的表皮下水疱起泡机理提供了新见识，而且为BP180和真皮-表皮锚固复合物的其他成分的生化结构和细胞生物学功能提供了新的见解。这些发现为这些自身免疫性和遗传性疾病的诊断和治疗工具的开发提出了新的方向。

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