Elastic fibres in health and disease
Elastic fibres in health and disease
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DOI：
10.1017/s146239940600007x
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发表时间：
2006-08-08
影响因子：
6.2
通讯作者：
Kielty, Cay M.
中科院分区：
文献类型：
--
作者：
Kielty, Cay M.
弹性纤维是一类主要的细胞外基质纤维，富含动态结缔组织，如动脉、肺、皮肤和韧带。它们的结构作用是赋予组织弹性反冲力和复原力。它们还充当细胞的重要粘附模板，并调节生长因子的可用性。弹性纤维主要结构成分（尤其是弹性蛋白、原纤维蛋白和 fibulin-5）的突变会导致严重的、常常危及生命的遗传性结缔组织疾病，例如马凡综合征、瓣膜上主动脉瓣狭窄和皮肤松弛。受损或老化的弹性组织的弹性纤维功能也经常受到损害。再生或改造弹性纤维和组织的能力仍然是一个重大挑战，需要更好地了解弹性纤维生物学和病理学的分子和细胞基础，以及调节其分子成分的时空表达和组装的能力。
Elastic fibres are a major class of extracellular matrix fibres that are abundant in dynamic connective tissues such as arteries, lungs, skin and ligaments. Their structural role is to endow tissues with elastic recoil and resilience. They also act as an important adhesion template for cells, and they regulate growth factor availability. Mutations in major structural components of elastic fibres, especially elastin, fibrillins and fibulin-5, cause severe, often life-threatening, heritable connective tissue diseases such as Marfan syndrome, supravalvular aortic stenosis and cutis laxa. Elastic-fibre function is also frequently compromised in damaged or aged elastic tissues. The ability to regenerate or engineer elastic fibres and tissues remains a significant challenge, requiring improved understanding of the molecular and cellular basis of elastic-fibre biology and pathology, and ability to regulate the spatiotemporal expression and assembly of its molecular components.