Lapiere J C, Hu L, Iwasaki T, Chan L S, Peavey C, Woodley D T
Department of Dermatology, Northwestern University School of Medicine, Chicago, IL 60611.
J Dermatol Sci. 1994 Oct;8(2):145-50. doi: 10.1016/0923-1811(94)90009-4.
Type VII collagen is the major component of anchoring fibrils, structures in human skin that mediate the adherence of the epidermis to the underlying dermis. Dystrophic forms of epidermolysis bullosa, a group of inherited mechanobullous disorders of the skin, are linked to the type VII collagen gene. Several mutations in the recessive form of this inherited disorder have been delineated. In this study, we mapped the epitopes of two commercially available monoclonal antibodies (clone I, 185 and LH 7.2) within the amino-terminal, non-collagenous domain of type VII (anchoring fibril) collagen. The precise localizations of the epitopes for these two monoclonal antibodies which are widely used to diagnose dystrophic epidermolysis bullosa, will be useful for the confirmation of gene mutations at the protein level.
VII型胶原蛋白是锚定原纤维的主要成分，锚定原纤维是人类皮肤中的结构，介导表皮与下方真皮的粘附。大疱性表皮松解症的营养不良形式是一组遗传性皮肤机械性大疱病，与VII型胶原蛋白基因有关。这种遗传性疾病的隐性形式中的几种突变已被确定。在本研究中，我们绘制了两种市售单克隆抗体（克隆I、185和LH 7.2）在VII型（锚定原纤维）胶原蛋白的氨基末端非胶原结构域内的表位。这两种广泛用于诊断营养不良性大疱性表皮松解症的单克隆抗体表位的精确定位，将有助于在蛋白质水平上确认基因突变。
