Proprotein convertase 2

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PCSK2
Identifiers
Aliases PCSK2, NEC 2, NEC-2, NEC2, PC2, SPC2, proprotein convertase subtilisin/kexin type 2
External IDs OMIM: 162151 MGI: 97512 HomoloGene: 37640 GeneCards: 5126
Genetically Related Diseases
Disease Name References
obesity
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_002594
NM_001201528
NM_001201529

NM_008792

RefSeq (protein)

NP_001188457.1
NP_001188458.1
NP_002585.2

NP_032818.1

Location (UCSC) Chr 20: 17.23 – 17.48 Mb Chr 2: 143.55 – 143.82 Mb
PubMed search [2] [3]
Wikidata
View/Edit Human View/Edit Mouse
proprotein convertase 2
Identifiers
EC number 3.4.21.94
CAS number 130960-94-0
Databases
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO

Proprotein convertase 2 (PC2) also known as prohormone convertase 2 or neuroendocrine convertase 2 (NEC2) is a serine protease and proprotein convertase PC2, like proprotein convertase 1 (PC1), is an enzyme responsible for the first step in the maturation of many neuroendocrine peptides from their precursors, such as the conversion of proinsulin to insulin intermediates. To generate the bioactive form of insulin (and many other peptides), a second step involving the removal of C-terminal basic residues is required; this step is mediated by carboxypeptidases E and/or D. PC2 plays only a minor role in the first step of insulin biosynthesis, but a greater role in the first step of glucagon biosynthesis compared to PC1. PC2 binds to the neuroendocrine protein named 7B2, and if this protein is not present, proPC2 cannot become enzymatically active. 7B2 accomplishes this by preventing the aggregation of proPC2 to inactivatable forms. The C-terminal domain of 7B2 also inhibits PC2 activity until it is cleaved into smaller inactive forms. Thus, 7B2 is both an activator and an inhibitor of PC2.

In humans, proprotein convertase 2 is encoded by the PCSK2 gene.[1] It is related to the bacterial enzyme subtilisin, and altogether there are 9 different subtilisin-like genes in mammals: furin, PACE4, PC4, PC5/6, PC7/8, PCSK9, and SKI1/S1P.

References[edit]

  1. ^ Seidah NG, Mattei MG, Gaspar L, Benjannet S, Mbikay M, Chrétien M (September 1991). "Chromosomal assignments of the genes for neuroendocrine convertase PC1 (NEC1) to human 5q15-21, neuroendocrine convertase PC2 (NEC2) to human 20p11.1-11.2, and furin (mouse 7[D1-E2] region)". Genomics 11 (1): 103–7. doi:10.1016/0888-7543(91)90106-O. PMID 1765368. 

Further reading[edit]

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