InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MBTPS1

Summary

InnateDB Protein

IDBP-44126.6

Last Modified

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

Gene Symbol

MBTPS1

Protein Name

membrane-bound transcription factor peptidase, site 1

Synonyms

PCSK8; S1P; SKI-1;

Species

Homo sapiens

Ensembl Protein

ENSP00000344223

InnateDB Gene

IDBG-44124 (MBTPS1)

Protein Structure

UniProt Annotation

Function

Serine protease that catalyzes the first step in the proteolytic activation of the sterol regulatory element-binding proteins (SREBPs). Other known substrates are BDNF, GNPTAB and ATF6. Cleaves after hydrophobic or small residues, provided that Arg or Lys is in position P4. Cleaves known substrates after Arg- Ser-Val-Leu (SERBP-2), Arg-His-Leu-Leu (ATF6), Arg-Gly-Leu-Thr (BDNF) and its own propeptide after Arg-Arg-Leu-Leu. Mediates the protein cleavage of GNPTAB into subunit alpha and beta, thereby participating in biogenesis of lysosomes. {ECO:0000269PubMed:21719679}.

Subcellular Localization

Endoplasmic reticulum membrane {ECO:0000269PubMed:9990022}; Single-pass type I membrane protein {ECO:0000269PubMed:9990022}. Golgi apparatus membrane {ECO:0000269PubMed:9990022}; Single-pass type I membrane protein {ECO:0000269PubMed:9990022}. Note=May sort to other organelles, including lysosomal and/or endosomal compartments.

Disease Associations

Tissue Specificity

Widely expressed.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 19 experimentally validated interaction(s) in this database.

Experimentally validated
Total	19 [view]
Protein-Protein	19 [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:0006629	lipid metabolic process
GO:0006987	activation of signaling protein activity involved in unfolded protein response
GO:0007040	lysosome organization
GO:0008203	cholesterol metabolic process
GO:0030968	endoplasmic reticulum unfolded protein response
GO:0042990	regulation of transcription factor import into nucleus
GO:0044267	cellular protein metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0000139	Golgi membrane
GO:0005788	endoplasmic reticulum lumen
GO:0005789	endoplasmic reticulum membrane
GO:0005794	Golgi apparatus
GO:0005795	Golgi stack
GO:0016021	integral component of membrane