InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PCSK1

Summary

InnateDB Protein

IDBP-34242.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PCSK1

Protein Name

proprotein convertase subtilisin/kexin type 1

Synonyms

BMIQ12; NEC1; PC1; PC3; SPC3;

Species

Homo sapiens

Ensembl Protein

ENSP00000308024

InnateDB Gene

IDBG-34238 (PCSK1)

Protein Structure

UniProt Annotation

Function

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 and insulin.

Subcellular Localization

Cytoplasmic vesicle, secretory vesicle. Note=Localized in the secretion granules.

Disease Associations

Proprotein convertase 1 deficiency (PC1 deficiency) [MIM:600955]: Characterized by obesity, hypogonadism, hypoadrenalism, reactive hypoglycemia as well as marked small- intestinal absorptive dysfunction It is due to impaired processing of prohormones. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 3 experimentally validated interaction(s) in this database.

Experimentally validated
Total	3 [view]
Protein-Protein	3 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004175	endopeptidase activity
GO:0004252	serine-type endopeptidase activity

Biological Process

GO:0006508	proteolysis
GO:0007267	cell-cell signaling
GO:0008152	metabolic process
GO:0016485	protein processing
GO:0016486	peptide hormone processing
GO:0043043	peptide biosynthetic process
GO:0044267	cellular protein metabolic process
GO:0050796	regulation of insulin secretion

Cellular Component

GO:0005615	extracellular space
GO:0005794	Golgi apparatus
GO:0030133	transport vesicle
GO:0034774	secretory granule lumen

Protein Structure and Domains

PDB ID

InterPro

IPR000209 Peptidase S8/S53 domain
IPR002884 Proprotein convertase, P
IPR008979 Galactose-binding domain-like
IPR009020 Proteinase inhibitor, propeptide
IPR015500 Peptidase S8, subtilisin-related
IPR022005 Prohormone convertase enzyme

PFAM

PF00082
PF01483
PF12177

PRINTS

PR00723

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P29120