Proprotein convertase 1
| Proprotein convertase 1 | ||
|---|---|---|
|
||
| according to PDB 1kn6 | ||
| Properties of human protein | ||
| Mass / length primary structure | 643 amino acids | |
| Cofactor | Calcium 2+ | |
| Identifier | ||
| Gene names | PCSK1 BMIQ12; NEC1; PC1; PC3; SPC3 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 3.4.21.93 , serine protease | |
| MEROPS | S08.072 | |
| Response type | Hydrolysis of a peptide bond | |
| Substrate | Proinsulin, Proopiomelanocortin, Prorenin, Proenkephalin, Prodynorphin, Prosomatostatin | |
| Occurrence | ||
| Parent taxon | Vertebrates | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 5122 | 18548 |
| Ensemble | ENSG00000175426 | ENSMUSG00000021587 |
| UniProt | P29120 | P63239 |
| Refseq (mRNA) | NM_000439 | NM_013628 |
| Refseq (protein) | NP_000430 | NP_038656 |
| Gene locus | Chr 5: 96.39 - 96.43 Mb | Chr 13: 75.09 - 75.13 Mb |
| PubMed search | 5122 |
18548
|
Proprotein convertase 1 (PC1) is an enzyme that is responsible for splitting off unnecessary protein components of many proteohormones and neuropeptides (the so-called post - translational modification ). For example, PC1 (together with PC 2 ) is necessary to produce insulin from proinsulin . PC1 is a serine protease found in many vertebrates . Mutations in the human PCSK1 - gene can lead to deficiency PC1 very rare, which is accompanied by severe metabolic disorders.
Substrates for PC1 include proinsulin, proopiomelanocortin , prorenin , proenkephalin , prodynorphin , prosomatostatin and oxytocin - neurophysin .
Individual evidence
- ↑ MEROPS entry
- ↑ UniProt P29120
- ↑ Jackson RS, Creemers JW, Farooqi IS, et al : Small-intestinal dysfunction accompanies the complex endocrinopathy of human proprotein convertase 1 deficiency . In: J. Clin. Invest. . 112, No. 10, November 2003, pp. 1550-60. doi : 10.1172 / JCI18784 . PMID 14617756 . PMC 259128 (free full text).