Anti-Human Fibulin-4 - Purified <i>in vivo </i>GOLD™ Functional Grade (F2200-GRP)

There are currently seven genes in the fibulin family (Fibulin-1, 2, 3, 4, 5, 6, and 7) that are associated with elastic fibers. The fibulins are widely distributed throughout the body and are more prominent in tissues rich in elastic fibers and are often associated with vasculature and elastic tissues. Fibulin-4 appears to be the most critical member of this family of proteins in modulating elastic-fiber formation because studies have shown that fibulin-4-null mice do not produce elastic fibers at all.1 This extracellular glycoprotein has a molecular weight of approximately 49 kD, and is not only considered a structural protein of the extracellular matrix, but also a regulatory factor required for normal elastin expression in human fibroblasts.2 Due to the regulatory role of Fibulin-4, it is thought that a pathophysiological link may exist between the modulation of fibulin-4 levels and diseases, such as WBS (Williams-Beuren syndrome), that are characterized by impaired elastic-fiber formation.1 Furthermore, fibulin-4 has been shown to interact with P53, a crucial homologous protein that prevents cancer formation, and thus functions as a tumor suppressor.3 One study indicated that colon tumors were found to have approximately 2-7-fold increases of fibulin-4 mRNA expression. This suggests that the dysregulated expression of the fibulin-4 gene is associated with human colon tumourigenesis.4