Fibulin-3

Fibulin-1 is a modular glycoprotein component of elastic extracellular matrix fibers, basement membranes and blood. It can bind calcium, Fibronectin, Laminin, Nidogen and Fibrinogen. Estrogen exposure to ovarian cancer cell lines can upregulate Fibulin-1. Fibulin-2 is abundant in heart, placenta and ovarian tissue and binds several components of the extracelllar matrix including aggrecan, versican and brevican. Fibulin-3, also known as EFEMP1, is a secreted protein. Defects in the gene for Fibulin-3 cause the autosomal dominant disease Doyne honeycomb retinal dystrophy (DHRD, also known as malattia leventinese) which is characterized by yellow-white deposits (drusen) that accumulate under the retinal pigment epithelium. Fibulin-3 is not present at the site of drusen formation in normal eyes. Fibulin-5 is an integrin-binding extracellular matrix protein that mediates endothelial cell adhesion.