Extracellular matrix fibrillins

Keene, D. R., Maddox, B. K, Kuo, H-J., Sakai, L. Y., and Glanville, R. W. (1991). Extraction of extensible beaded structure and their identification as extracellular matrix fibrillin-containing microfibrils./. Histochem. Cytochem. 39, 441-449. 

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... 

Kielty, C. M., Sherratt, M.J., Haston,J. L., Wess, T. J., and Shutdeworth, C. A. (2002a). Fibrillin-rich microfibrils Elastic biopolymers of the extracellular matrix. J. Muscle Res. Cell Motil. 23, 581-596. 

Elastic fibers form the network in skin and cardiovascular tissue (elastic arteries) that is associated with elastic recovery. Historically the recovery of skin and vessel wall on removal of mechanical loads at low strains has been attributed to elastic fibers. Elastic fibers are composed of a core of elastin surrounded by microfibrils 10 to 15 nm in diameter composed of a family of glycoproteins recently termed fibrillins. Fibrillins are a family of extracellular matrix glycoproteins (MW about 350,000) containing a large number of cysteine residues (cysteine residues form disulfide crosslinks). Several members of the family have been described. The common molecular features include N and C terminal ends with 47 tandemly repeated epi-... 

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