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Structural and functional failure of fibrillin‑1 in human diseases (Review)

22 Dec 2017

Abstract excerpt

Fibrillins (FBNs) are key relay molecules that form the backbone of microfibrils in elastic and non‑elastic tissues. Interacting with other components of the extracellular matrix (ECM), these ubiquitous glycoproteins exert pivotal roles in tissue development, homeostasis and repair. In addition to mechanical support, FBN networks also exhibit regulatory activities on growth factor signalling, ECM formation, cell...

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PMID: 29286095
DOI: 10.3892/ijmm.2017.3343

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