Elastic fibre-matrix interactions important for tissue development, morphology and function

许多组织的性质由细胞外基质中结构蛋白的类型、比例和组织控制。这些不同蛋白质之间的分子相互作用对于正常组织的强度、形式和功能是必不可少的。我们正在研究弹性纤维的蛋白质成分与周围基质的结构分子（包括胶原蛋白、蛋白聚糖和基底膜成分）的相互作用。目前，我们的知识，这些相互作用是有限的，即使他们是必不可少的锚定弹性纤维在柔顺组织，如动脉壁，肺和皮肤的拉伸过程中。此外，几种弹性纤维相关蛋白似乎在非弹性组织如骨和角膜的基质中很重要。该项目将继续增加我们对不同结缔组织正常发育和功能的基本了解。这一知识将反过来导致对以下机制的更深入的阐明：a）在主要疾病过程中的组织功能丧失，如动脉粥样硬化、血管动脉瘤、肺气肿和UV诱导的皮肤损伤，和B）先天性疾病中的发育异常，如马凡氏综合征和各种角膜营养不良。

The properties of many tissues are controlled by the type, ratio and organisation of structural proteins laid down into extracellular matrices. Molecular interactions between these different proteins are essential for normal tissue strength, form and function. We are investigating the interactions of protein components of elastic fibres, with structural molecules of the surrounding matrix including collagens, proteoglycans and basement membrane components. At present our knowledge of these interactions is limited even though they are essential for anchorage during stretching of elastic fibres in compliant tissues such as arterial walls, lung and skin. In addition several of the elastic fibre-associated proteins appear to be important in the matrix of non-elastic tissues such as bone and cornea. The project will continue to increase our fundamental understanding of the normal development and function of different connective tissues. This knowledge will, in turn, lead to much greater elucidation of the mechanisms leading to a) loss of tissue function in major disease processes such as atherosclerosis, blood vessel aneurysms, pulmonary emphysema and UV-induced skin damage, and b) developmental abnormalities in congenital disorders such as Marfan syndrome and a variety of corneal dystrophies.