Pathomechanism for increased ectodomain shedding of collagen XVIIby targeting coiled-coils

胶原XVII （COL17）是大疱性类天疱疮的主要自身抗原。COL17的近膜NC16A结构域包含主要的表位，其中存在潜在的线圈。本研究的目的是解决盘绕线圈的病理机制。实验表明，螺旋线圈是COL17形成三螺旋结构的重要动机；此外，当缺失折叠的COL17产生时，蛋白酶可以靶向这些动机。因此，该结构域对折叠后的COL17的质量检测具有新的重要作用。

Collagen XVII (COL17) is the major autoantigen for bullous pemphigoid. Juxtamembranous NC16A domain of COL17 contains major epitopes, in which potential coiled-coil exits. The aim of this study is to address the pathomechanism of the coiled-coils. The experiments disclosed that coiled-coils are essential motives for COL17 to form triple-helical structure; in addition the motived can be targeted by proteases when miss-folded COL17 are produced. Thus, the domain has a novel vital role for quality checking of folded COL17.