LTBP4 : latent transforming growth factor beta binding protein 4
Description
The LTBP4 (latent transforming growth factor beta binding protein 4) is a protein-coding gene located on chromosome 19.
The LTBP4 gene provides instructions for making a protein that plays several important roles in the extracellular matrix. This protein regulates the activity of a protein called transforming growth factor beta-1 (TGFβ-1) by attaching it to the extracellular matrix. TGFβ-1 is inactive when stored in the extracellular matrix and becomes active when released. Once active, TGFβ-1 triggers chemical signals that direct cell functions during growth and development, and also regulate the formation of the extracellular matrix. LTBP4 also has a critical role in the formation of elastic fibers in the extracellular matrix. Elastic fibers are bundles of proteins that provide strength and flexibility to connective tissues, which support organs and tissues such as the skin, lungs, heart, ligaments, and blood vessels.
LTBP4 plays a crucial role in regulating the activity of transforming growth factor beta (TGF-β) by keeping it inactive while stored in the extracellular matrix. It achieves this by forming a complex with the latency-associated peptide (LAP) of TGF-β, specifically through disulfide bonds. This complex ensures the proper activation of TGF-β, which is triggered by integrins.
LTBP4 is also known as ARCL1C, LTBP-4, LTBP4L, LTBP4S.
