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.

中文翻译：

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原纤维蛋白和微原纤维结合蛋白在弹性蛋白和弹性纤维组装中的作用。


原纤维蛋白是一种大型的进化古老的细胞外糖蛋白，它组装形成珠状微原纤维，是大多数细胞外基质的重要组成部分。原纤维蛋白微纤维具有特定的生物力学特性，赋予动物组织有限的弹性，这是大血管、皮肤和肺部持久功能的基本特征。它们还形成弹性蛋白沉积的模板，并为微原纤维-弹性蛋白结合蛋白在弹性纤维组装中相互作用提供平台。除了其结构作用外，原纤维蛋白微原纤维还通过整合素和多聚糖受体介导细胞信号传导，并且微原纤维将转化生长因子 (TGF)β 家族生长因子隔离在基质内，以提供对体内平衡和重塑至关重要的组织储存。