Collagen XXII: A novel collagen of basement membranes and other tissue junctions

XXII 型胶原蛋白：基底膜和其他组织连接处的一种新型胶原蛋白

• 批准号: 5394426
• 负责人: Professor Dr. Manuel Koch
• 金额: --
• 依托单位: Institut für experimentelle Zahnmedizin und orale muskuloskeletale Biologie
• 依托单位国家: 德国
• 项目类别: Priority Programmes
• 财政年份: 2002
• 资助国家: 德国
• 起止时间: 2001-12-31 至 2007-12-31
• 项目状态: 已结题
• 来源: https://gepris.dfg.de/gepris/projekt/5394426?language=en
• 关键词: Collagen; XXII; novel; collagen; basement

The collagen family of proteins is essential for maintaining the integrity of tissues. The functions of these proteins have been clearly illustrated by a wide spectrum of human diseases resulting from mutations in collagen genes. In the last few years, seven novel collagens have been identified in the human genome databases. Here, we propose structural and functional characterization of one of them, collagen XXII, which is localized at the basement membranes of myotendinous junctions and hair follicles, and to certain other tissue junctions in muscle, heart, eye and cartilage. We will produce collagen XXII as a recombinant protein and investigate its biosynthesis, structure, interaction partners and regulation. To elucidate the functions of collagen XXII, a generation of knockout mice has been initiated. In parallel, the involvement of this collagen in human pathology will be addressed. The restricted tissue expression in several organs predicts a role for collagen XXII in inherited or acquired human syndromes, including hair disorders or muscular dystrophies, or as an autoantigen in autoimmune diseases with multiorgan symptoms.

胶原蛋白家族对于维持组织的完整性至关重要。由胶原蛋白基因突变引起的多种人类疾病清楚地说明了这些蛋白质的功能。在过去的几年里，人类基因组数据库中发现了七种新型胶原蛋白。在这里，我们提出了其中一种胶原蛋白 XXII 的结构和功能特征，它位于肌腱连接处和毛囊的基底膜上，以及肌肉、心脏、眼睛和软骨中的某些其他组织连接处。我们将生产 XXII 型胶原蛋白作为重组蛋白，并研究其生物合成、结构、相互作用伙伴和调控。为了阐明 XXII 胶原蛋白的功能，已经开始培育一代基因敲除小鼠。同时，还将讨论这种胶原蛋白与人类病理学的关系。多个器官中有限的组织表达预示着 XXII 胶原蛋白在遗传性或获得性人类综合征（包括毛发疾病或肌肉营养不良）中的作用，或作为自身抗原在具有多器官症状的自身免疫性疾病中的作用。