Elastin

Elastin is a key extracellular matrix protein that is roughly a 1000 times more flexible than collagens. The main function of elastin is to provide elasticity and resilience to extensible tissues like the lungs, aorta, and skin. The precursor of elastin is tropoelastin, which is primarily produced in fibroblasts, smooth muscle cells, chondrocytes, and endothelial cells before being cross-linked to form mature elastin. Mutations in the elastin gene may lead to diseases such as Williams–Beuren syndrome, cutis laxa, and supravalvular aortic stenosis. Elastin degradation can release bioactive fragments with diverse signaling properties that can drive disease progression, as seen in cancer and emphysema. In addition, elastin degradation has been linked to chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, and cardiovascular disease. Several important and well-described biomarkers of elastin degradation are available.