Fibrillin microfibrils

Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and non elastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1—the principal structural component of microfibrils—cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm beads-on-a-string appearance the molecular basis of their assembly and... 

Charbonneau, N. L., Ono, R. N., Corson, G. M., Keene, D. R., and Sakai, L. Y. (2004). Fine tuning of growth factor signals depends on fibrillin microfibril networks. Birth Defects Res. Part C Embryo Today 72, 37-50. 

Kielty, C. M., Woolley, D. E., Whittaker, S. P., and Shutdeworth, C. A. (1994). Catabolism of intact fibrillin microfibrils by neutrophil elastase, chymotrypsin and trypsin. FEBS Lett. 351, 85-89. 

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