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Dissecting the Fibrillin Microfibril: Structural Insights into Organization and Function

Authors :
Penny A. Handford
Sacha A. Jensen
Ian B. Robertson
Source :
Structure. 20(2):215-225
Publication Year :
2012
Publisher :
Elsevier BV, 2012.

Abstract

Force-bearing tissues such as blood vessels, lungs, and ligaments depend on the properties of elasticity and flexibility. The 10 to 12 nm diameter fibrillin microfibrils play vital roles in maintaining the structural integrity of these highly dynamic tissues and in regulating extracellular growth factors. In humans, defective microfibril function results in several diseases affecting the skin, cardiovascular, skeletal, and ocular systems. Despite the discovery of fibrillin-1 having occurred more than two decades ago, the structure and organization of fibrillin monomers within the microfibrils are still controversial. Recent structural data have revealed strategies by which fibrillin is able to maintain its architecture in dynamic tissues without compromising its ability to interact with itself and other cell matrix components. This review summarizes our current knowledge of microfibril structure, from individual fibrillin domains and the calcium-dependent tuning of pairwise interdomain interactions to microfibril dynamics, and how this relates to microfibril function in health and disease.

Details

ISSN :
09692126
Volume :
20
Issue :
2
Database :
OpenAIRE
Journal :
Structure
Accession number :
edsair.doi.dedup.....f0d54524597a8d0ad739e74b0dde202a
Full Text :
https://doi.org/10.1016/j.str.2011.12.008