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VII型胶原蛋白是表皮基底膜的正常组成部分,在隐性营养不良性大疱性表皮松解症中其表达会发生改变。

Type VII collagen is a normal component of epidermal basement membrane, which shows altered expression in recessive dystrophic epidermolysis bullosa.

作者信息

Leigh I M, Eady R A, Heagerty A H, Purkis P E, Whitehead P A, Burgeson R E

机构信息

Experimental Dermatology Laboratory, London Hospital, England.

出版信息

J Invest Dermatol. 1988 May;90(5):639-42. doi: 10.1111/1523-1747.ep12560795.

Abstract

The murine monoclonal antibody LH 7:2, which reacts with the basement membrane of stratified squamous epithelia including epidermis, has been characterized biochemically and shown to bind to part of the type VII collagen molecule. Immunoblotting reveals that the antibody binding site lies in the non-helical carboxy terminal region of the type VII collagen dimer and immunoelectron microscopy shows that the epitope is within the lamina densa of the basement membrane. Loss of LH 7:2 binding in the hereditary blistering disease recessive dystrophic epidermolysis bullosa suggests that inadequate synthesis or excessive breakdown of type VII collagen may form the biologic basis for the disease.

摘要

鼠单克隆抗体LH 7:2可与包括表皮在内的复层鳞状上皮的基底膜发生反应,已对其进行了生化特性鉴定,结果表明它可与VII型胶原分子的一部分结合。免疫印迹显示,该抗体结合位点位于VII型胶原二聚体的非螺旋羧基末端区域,免疫电子显微镜显示该表位位于基底膜的致密层内。遗传性水疱病隐性营养不良型大疱性表皮松解症中LH 7:2结合的丧失表明,VII型胶原合成不足或过度分解可能构成该疾病的生物学基础。

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