Collagenous transmembrane proteins: collagen XVII as a prototype
Collagenous transmembrane proteins: collagen XVII as a prototype
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DOI：
10.1016/s0945-053x(03)00051-9
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发表时间：
2003-06-01
期刊：
影响因子：
6.9
通讯作者：
Bruckner-Tuderman, L
中科院分区：
文献类型：
--
作者：
Franzke, CW;Tasanen, K;Bruckner-Tuderman, L
胶原跨膜蛋白是一类新兴的生物多用途分子，它既是细胞表面受体又是基质分子。这7组成员具有有趣的结构相似性：它们是II型取向的整体膜蛋白，在细胞外c端具有一个或多个胶原结构域；由非胶原拉伸穿插，赋予外畴结构灵活性。紧挨着细胞膜细胞外表面的一个保守的卷曲序列（连接域）可能是每个胶原蛋白三聚化和三螺旋折叠的细胞核。有趣的是，至少其中一些分子的外结构域以蛋白质水解的方式从细胞表面脱落，将较短形式的胶原蛋白释放到细胞外基质中。胶原跨膜蛋白在许多不同的组织和细胞中表达，并参与广泛的生物学功能，从上皮和神经细胞的粘附，到形态发生过程中上皮-间充质相互作用，再到宿主对微生物制剂的防御。一些小组成员参与遗传和获得性人类疾病的分子病理学研究，包括大疱性表皮松解症、外胚层发育不良、大疱性类天疱疮或阿尔茨海默病。广泛研究的成员是胶原XVII，一种角化细胞表面蛋白，它将表皮附着在皮肤的基底膜上。本文对目前已知的胶原跨膜蛋白的结构和功能进行了综述，并对作为该蛋白“原型”的XVII胶原及其生物学和病理生理进行了阐述。(C) 2003 Elsevier Science B.V./国际基质生物学学会。版权所有。
Collagenous transmembrane proteins are an emerging group of biologically versatile molecules which function as both cell surface receptors and matrix molecules. The seven group members have interesting structural similarities: they are integral membrane proteins in type II orientation and have one or more collagenous domains in the extracellular C-terminus; interspersed by non-collagenous stretches which confer structural flexibility to the ectodomain. A conserved coiled-coil sequence (linker domain) immediately adjacent to the extracellular face of the cell membrane presumably serves as a nucleus for trimerization and triple-helix folding of each collagen. Intriguingly, the ectodomains of at least some of these molecules are proteolytically shed from the cell surface, releasing a shorter form of the collagen into the extracellular matrix. Collagenous transmembrane proteins are expressed in many different tissues and cells, and are involved in a broad spectrum of biological functions, reaching from epithelial and neural cell adhesion, and epithelial-mesenchymal interactions during morphogenesis to host defense against microbial agents. Several group members are involved in the molecular pathology of genetic and acquired human diseases including epidermolysis bullosa, ectodermal dysplasia, bullous pemphigoid or Alzheimer disease. An extensively investigated member is collagen XVII, a keratinocyte surface protein, which attaches the epidermis to the basement membrane in the skin. In this review, the structure and functions of the currently known collagenous transmembrane proteins are summarized and, as a `prototype' of the group, collagen XVII and its biology and pathophysiology are delineated. (C) 2003 Elsevier Science B.V./International Society of Matrix Biology. All rights reserved.