Molecular mechanism of elastic fiber assembly.

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Elastic fiber is abundant in elastic tissues such as arteries, lungs and skin, and provides elasticity to these tissues. Aging-associated elastic fiber degeneration or fragmentation causes arteriosclerosis, emphysema and loose skin. It is important to uncover the molecular mechanism of elastogenesis for the future elastic fiber regeneration therapy. We previously reported that a secreted protein Fibulin-5 is essential for elastic fiber assembly. In this study, we studied the role for Fibulin-4, a member of the same protein family, in elastogenesis. Smooth muscle-specific knockout of the mouse Fbln4 gene resulted in degenerated elastic laminae in aortae and aneurysm. We also found that Fibulin-4 directly interacts with lysyl oxidase and tropoelastin, thereby tethering the enzyme to its substrate.