EFEMP2 : EGF containing fibulin extracellular matrix protein 2
Description
The EFEMP2 (EGF containing fibulin extracellular matrix protein 2) is a protein-coding gene located on chromosome 11.
The EFEMP2 gene provides instructions for making a protein called EGF-containing fibulin extracellular matrix protein 2, which is also known as fibulin-4. This protein is part of a group of proteins called fibulins. Fibulins have a variety of functions in the extracellular matrix, which is the intricate lattice of proteins and other molecules that forms in the spaces between cells. Little is known about the function of fibulin-4 in the extracellular matrix. It appears to play a critical role in the assembly of elastic fibers, which are slender bundles of protein that provide strength and flexibility to connective tissue (tissue that supports the body's joints and organs). Fibulin-4 is found in tissues and organs that are rich in elastic fibers, including the blood vessels, heart valves, lungs, and skin.
EFEMP2 plays a crucial role in the formation of elastic fibers in tissues and in the formation of connections between elastic laminae and smooth muscle cells in the aorta. This contributes to the terminal differentiation and maturation of smooth muscle cells, and maintains the mechanical properties and wall integrity of the aorta. Additionally, EFEMP2 is involved in regulating collagen fibril assembly by activating LOX, leading to the cross-linking of collagen and elastin. It also promotes the aggregation of elastin (ELN) and its deposition on microfibrils, while simultaneously regulating ELN cross-linking through interactions with LOX. EFEMP2 adheres to cells via heparin binding in a calcium-dependent manner, regulating vascular smooth muscle cell proliferation through angiotensin signaling.
EFEMP2 is also known as ARCL1B, FBLN4, MBP1, UPH1.
