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FBN1, also named as FBN, belongs to the fibrillin family. Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Defects in FBN1 are a cause of Marfan syndrome (MFS), isolated ectopia lentis (EL), Weill-Marchesani syndrome autosomal dominant (ADWMS), Shprintzen-Goldberg craniosynostosis syndrome (SGS) or MASS syndrome. This antibody is specific to FBN1.