ECM1 Recombinant Rabbit Monoclonal Antibody [JE54-17]

ECM1 (extracellular matrix protein 1), also known as secretory component p85, is a secreted glycoprotein that is essential for the proper structure and function of the skin. It is widely expressed and localizes to the extracellular matrix. ECM1 binds to a variety of extracellular matrix components, including Perlecan, Fibulin and matrix metalloproteinase-9 (MMP-9), and participates in the structural organization of the dermis. In addition, ECM1 enhances the association of Collagen Type IV with Laminin 332 suggesting that it is a key player in interstitial dermis and the dermal-epidermal junction. Mutations in the gene encoding ECM1 result in the autosomal recessive disorder lipoid proteinosis (LiP). LiP is characterized by hyalinization of the dermis and reduplication of the basement membrane of the skin. LiP patients exhibit thickening of the skin and mucosae. Four splice variants (known as ECM1a-ECM1d) exist for ECM1.