Receptor for advanced glycation end product (RAGE) is a 35 kDa transmembrane receptor of the immunoglobulin super family. Its name comes from its ability to bind advanced glycation endproducts (AGE). In view of its inflammatory function in innate immunity and its ability to detect a class of ligands through a common motif, RAGE is often referred to as a pattern recognition receptor. RAGE has been linked to several chronic diseases, such as atherosclerosis, peripheral vascular disease, myocardial infarction, congestive heart failure, diabetes, Alzheimer’s disease and so on. RAGE is expressed at the highest levels in the lung compared to other tissues, in particular in alveolar epithelial type I cells, and is lost in Idiopathic pulmonary fibrosis (IPF) indicating that expression and regulation of RAGE in the pulmonary system differs from that in the vascular system. Blockade/knockdown of RAGE resulted in impaired cell adhesion, and increased cell proliferation and migration.