Wellcome Centre for Cell-Matrix Research, Division of Cell-Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester M13 9PT, UK.
Wellcome Centre for Cell-Matrix Research, Division of Cell-Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester M13 9PT, UK.
Matrix Biol. 2019 Nov;84:17-30. doi: 10.1016/j.matbio.2019.06.006. Epub 2019 Jun 18.
Fibrillin is a large evolutionarily ancient extracellular glycoprotein that assembles to form beaded microfibrils which are essential components of most extracellular matrices. Fibrillin microfibrils have specific biomechanical properties to endow animal tissues with limited elasticity, a fundamental feature of the durable function of large blood vessels, skin and lungs. They also form a template for elastin deposition and provide a platform for microfibril-elastin binding proteins to interact in elastic fibre assembly. In addition to their structural role, fibrillin microfibrils mediate cell signalling via integrin and syndecan receptors, and microfibrils sequester transforming growth factor (TGF)β family growth factors within the matrix to provide a tissue store which is critical for homeostasis and remodelling.
纤维连接蛋白是一种大型的进化古老的细胞外糖蛋白，它组装成珠状微纤维，是大多数细胞外基质的重要组成部分。纤维连接蛋白微纤维具有特定的生物力学特性，为动物组织赋予有限的弹性，这是大血管、皮肤和肺等持久功能的基本特征。它们还为弹性蛋白的沉积提供了一个模板，并为微纤维-弹性蛋白结合蛋白在弹性纤维组装中相互作用提供了一个平台。除了它们的结构作用外，纤维连接蛋白微纤维还通过整合素和 syndecan 受体介导细胞信号转导，并且微纤维将转化生长因子 (TGF)β 家族生长因子隔离在基质中，为组织储存提供了一个关键的平衡和重塑的组织库。
