Recombinant Human Proprotein Convertase 1/PCSK1 Protein, CF

The human PCSK1 gene encodes Proprotein Convertase 1 (PC1), which is also known as PC3 (1, 2). As a serine protease of the furin/PC family, the deduced amino acid sequence of human PC1 consists of a signal peptide (residues 1 to 27), a pro peptide (residue 28 to 110), and a mature chain (residues 111 to 753). Autocatalytic processing results in the removal of the pro peptide as well as a region from the C-terminus, which leads to the production of the most active 66 kDa form (3). The purified rhPC1 consists of residues 111 to 617, is active in the assay described above and most likely corresponds to the 66 kDa form. Found almost exclusively in endocrine and neural cells and stored as the 66 kDa form in dense-core secretory vesicles, PC1 is capable of processing several endocrine and neural prohormones, such as opiomelanocortin, somatostatin, enkephalin, insulin, dynorphin and thyrotropin-releasing hormone (3). Indeed, obesity and impaired prohormone processing have been associated with mutations in the human PCSK1 gene (4).