InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PCSK1N

Summary

InnateDB Protein

IDBP-64218.4

Last Modified

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

Gene Symbol

PCSK1N

Protein Name

proprotein convertase subtilisin/kexin type 1 inhibitor

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000218230

InnateDB Gene

IDBG-64216 (PCSK1N)

Protein Structure

UniProt Annotation

Function

May function in the control of the neuroendocrine secretory pathway. Proposed be a specific endogenous inhibitor of PCSK1. ProSAAS and Big PEN-LEN, both containing the C-terminal inhibitory domain, but not the further processed peptides reduce PCSK1 activity in the endoplasmic reticulum and Golgi. It reduces the activity of the 84 kDa form but not the autocatalytically derived 66 kDa form of PCSK1. Subsequent processing of proSAAS may eliminate the inhibition. Slows down convertase-mediated processing of proopiomelanocortin and proenkephalin. May control the intracellular timing of PCSK1 rather than its total level of activity. The function of the processed secreted peptides is not known (By similarity). {ECO:0000250}.

Subcellular Localization

Secreted {ECO:0000250}. Golgi apparatus, trans-Golgi network {ECO:0000250}. Note=A N-terminal processed peptide, probably Big SAAS or Little SAAS, is accumulated in cytoplasmic protein tau deposits in frontotemporal dementia and parkinsonism linked to chromosome 17 (Pick disease), Alzheimer disease and amyotrophic lateral sclerosis-parkinsonism/dementia complex 1 (Guam disease). {ECO:0000269PubMed:12914799, ECO:0000269PubMed:14746899}.

Disease Associations

Tissue Specificity

Expressed in brain and pancreas. {ECO:0000269PubMed:10632593}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.

Experimentally validated
Total	4 [view]
Protein-Protein	0
Protein-DNA	4 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004866	endopeptidase inhibitor activity
GO:0004867	serine-type endopeptidase inhibitor activity
GO:0005102	receptor binding

Biological Process

GO:0002021	response to dietary excess
GO:0007218	neuropeptide signaling pathway
GO:0009409	response to cold
GO:0010951	negative regulation of endopeptidase activity
GO:0016486	peptide hormone processing