PCPE-2 Activators

PCPE-2, or Procollagen C-Proteinase Enhancer 2, is a protein encoded by the PCOLCE2 gene in humans. This protein is known to play a key role in the biosynthesis and assembly of collagen fibrils, which are integral components of the extracellular matrix (ECM) found in connective tissues.The primary function of PCPE-2 is to enhance the enzymatic cleavage of procollagen by C-proteinases (also known as BMP-1 or bone morphogenetic protein-1 and related proteases), which is a critical step in collagen maturation. Procollagen is a precursor form of collagen that contains additional peptide extensions at both the N-terminal and C-terminal ends. These extensions, called propeptides, must be cleaved to allow the collagen molecules to assemble into the characteristic triple-helical structure of mature collagen fibrils.

PCPE-2 specifically binds to the C-propeptide region of procollagen and enhances the activity of proteinases that cleave this region, thus facilitating collagen fibrillogenesis. The efficiency of this process is vital for the proper formation of collagen fibrils and, consequently, for the structural integrity of tissues such as skin, bone, cartilage, tendons, and ligaments.Mutations or dysregulation in the PCOLCE2 gene, and thus in the PCPE-2 protein, can lead to defects in collagen fibril formation and are associated with various connective tissue disorders. Understanding the function and regulation of PCPE-2 is important for insights into the fundamental processes of tissue development and repair.