- OMIM: 162 150
- UniProt: P29120
Proprotein convertase 1 (PC1 ) is an enzyme ( the so-called post-translational modification ), for the removal of unnecessary protein constituents of many proteohormones and neuropeptides responsible. For example, PC1 ( PC along with 2) required to produce insulin from proinsulin. When PC1 is a serine protease that is found in many vertebrates. Mutations in the human PCSK1 gene can lead to very rare PC1 deficiency, which is associated with severe metabolic disorders.
Substrates for PC1 include proinsulin, proopiomelanocortin, prorenin, proenkephalin, prodynorphin, Prosomatostatin and oxytocin neurophysin.