The receptor for advanced glycosylation end products (RAGE) is a 35 kDa immunoglobulin superfamily transmembrane receptor that derives its name from the ability to bind to advanced glycosylation end products. Given its inflammatory function in innate immunity and its ability to recognize a class of ligands through a common motif, RAGE is often considered a class I recognition receptor.RAGE has been associated with a number of chronic diseases such as atherosclerosis, peripheral vascular disease, myocardial infarction, congestive heart failure, diabetes mellitus, and Alzheimer’s disease. Compared with other tissues, RAGE is most highly expressed in the lungs, especially in type I alveolar epithelial cells, and not in idiopathic pulmonary fibrosis (IPF), suggesting that RAGE is differently expressed and regulated in the pulmonary lineage than in the cardiovascular system. Blockade/knockdown of RAGE results in impaired cell adhesion and increased cell proliferation and migration.