Fibrillin Antibody [11C1.3] (Biotin)

Background:

The fibrillins are extracellular matrix glycoproteins which occur as a major component of a subset of connective tissue microfibrils. These microfibrils have a beaded appearance and a cross-sectional diameter of 10-12 nm. In elastic tissues, these structures are thought to provide the scaffold onto which elastin is assembled to form elastic fibers, although their function in non-elastic tissues is unclear. There is an aberrant expression of fibrillin in Marfan syndrome. The epitope for the monoclonal antibody 11C1.3 has been found to be within exons 18-20 (a.a. 723-909).