Entrez Summary: This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to subcellular compartments where a second autocatalytic even takes place and the catalytic activity is acquired. The protease is packaged into and activated in dense core secretory granules and expressed in the neuroendocrine system and brain. This gene encodes one of the seven basic amino acid-specific members which cleave their substrates at single or paired basic residues. It functions in the proteolytic activation of polypeptide hormones and neuropeptides precursors. Mutations in this gene have been associated with susceptibility to obesity and proprotein convertase 1/3 deficiency. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene [provided by RefSeq, Jan 2014].
UniProt Summary: Involved in the processing of hormone and other protein precursors at sites comprised of pairs of basic amino acid residues. Substrates include POMC, renin, enkephalin, dynorphin, somatostatin, insulin and AGRP. {ECO:0000250|UniProtKB:P63239}.
Pfam DomainsGO Terms
Pfam Domains
P proprotein
Proho convert
Peptidase S8
GO Terms
peptide hormone processing
transport vesicle
secretory granule lumen
protein processing
serine-type endopeptidase activity
hormone metabolic process
protein maturation
neuron projection
peptide biosynthetic process
amide biosynthetic process
peptide metabolic process
regulation of hormone levels
cellular amide metabolic process
identical protein binding
cell-cell signaling
proteolysis
organonitrogen compound biosynthetic process
extracellular space
cellular nitrogen compound biosynthetic process
membrane
gene expression
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)