P50454

HSP 47, also known as collagen 1, serpinh1, collagen-binding protein 1 (CBP1) and gp46, is expressed in smooth muscle cells, specifically in the interstitial space between tubules, vascular smooth muscle and medullary rays. It is expressed constitutively in cells that synthesize collagen and is involved in Collagen Type I biosynthesis. HSP 47 plays a vital role in folding and assembling collagen. A procollagen-specific molecular chaperone, HSP 47 resides in the endoplasmic reticulum of procollagen-producing cells and is essential for secretion of procollagen from cells. After insult, it acts as a stress response molecule to sequester abnormal procollagen. HSP 47 synthesis is induced by TGFβ and IL-1β.