<p>This entry represents fibrillin, a structural component of connective tissue microfibrils that binds calcium. Fibrillin molecules are very large (&gt;300 kDa) complex multidomain gylcoproteins. Fibrillin microfibrils endow connective tissue with long-range elasticity, being particularly abundant in elastic tissues, such as the lung, and also in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Though there are three fibrillin genes with distinct, but overlapping, tissue distributions, fibrillin-1 is the major structural component of microfibrils. Mutations in the fibrillin-1 gene cause Marfan syndrome, a hereditable disease which can cause severe heart, eye and skeletal defects. For an in-depth review see [<cite idref="PUB00028054"/>].</p> Fibrillin