Recombinant Human Fibulin 3 Protein, CF

Human Fibulin-3, also called EGF-containing fibulin-like extracellular matrix protein 1 (EFEMP-1), is an approximately 57 kDa member of the fibulin family of proteins (1). It was originally identified in fibroblasts from a Werner syndrome patient with premature aging (2). Along with Fibulin-4 and -5, Fibulin-3 is referred to as a class II, or short fibulin (3). Fibulin-3 contains five calcium-binding EGF-like motifs and a fibulin-type C terminus (1). Human Fibulin-3 shares 95% and 93% amino acid sequence identity with mouse and rat Fibulin-3, respectively. It has been shown to be susceptible to MMP-mediated proteolysis (4). Fibulin-3 appears to be involved in the proper function of elastic tissues. Knockout mice are viable, but display disrupted elastic fibers in fascia connective tissue of the body wall (5, 6). In humans, Fibulin-3 mutations have been associated with Doyne honeycomb retinal dystrophy (7). Its expression is up-regulated in late, metastatic stages of carcinomas, suggesting that it may play a role in cancer (8, 9). Fibulin-3 promotes the growth of gliomas and may have a pro-angiogenic role in this cancer type (10, 11). In contrast, Fibulin-3 has been shown to negatively modulate the invasiveness of lung cancer cells and inhibit self-renewal of lung cancer stem cells (12-14).