特異性：Human and bovine fibrillin (Mr 350 kDa); other species not tested 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.^1 In immunohistochemical staining of paraformaldehyde-fixed human skin, mAb 11C1.3 labels the elastic framework and more specifically the oxytalantic network close to the epidermis. The antibody also exhibits discrete staining of normal fibroblasts in culture and of extracellular (elastin) fibrils in a variety of tissues. ^2,3 There is an aberrant expression of fibrillin in Marfan syndrome. ^4
