epitope corresponding to amino acids 2726-2834 of fibrillin-1 of human origin
recommended for detection of fibrillin-1 of mouse, rat and human origin by WB, IP, IF and ELISA; also reactive with additional species, including equine, canine, bovine and porcine
fibrillin-1 Background Information The fibrillin family of proteins, including fibrillin 1 (Fbn-1) and fibrillin 2 (Fbn-2), are integral components of a distinct subset of extracellular microfibrils (1,2). Microfibrils are found in elastic tissues where they facilitate elastic fiber formation and in nonelastic tissue where they support the association of the epithelial cells with the interstitial matrix (3). Characteristic of the fibrillin proteins are the epidermal growth factor (EGF)-like motifs which contain a consensus sequence for calcium binding. This calcium association may be critical for protein-protein interactions and stabilization of the microfibrils (4). Mutations of the FBN1 gene have been shown to result in Marfan syndrome, a disease characterized by abnormal synthesis, secretion and matrix deposition of fibrillin (5). FBN2 is also linked to a rare, yet similiar skeletal disorder, congenital contractural arachnodactyly (6).
