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.

蛋白质的胶原蛋白家族对于维持组织的完整性至关重要。这些蛋白质的功能已通过胶原基因突变引起的广泛的人类疾病明确说明。在过去的几年中，在人类基因组数据库中发现了七个新型胶原蛋白。在这里，我们提出了其中一个的结构和功能表征，即胶原蛋白XXII，该胶原蛋白XXII位于肌齿状连接和毛囊的地下膜中，以及在肌肉，心脏，眼睛和软骨中的某些其他组织连接处。我们将产生胶原蛋白作为重组蛋白，并研究其生物合成，结构，相互作用伙伴和调节。为了阐明胶原蛋白XXII的功能，已经启动了一代敲除小鼠。同时，将解决该胶原蛋白在人类病理学中的参与。在几个器官中的受限组织表达预测胶原蛋白XXII在遗传或获得的人类综合征中的作用，包括毛发疾病或肌肉发育不全，或作为自身免疫性疾病的自身抗原具有多器官症状。