Names & Taxonomy
- Uniprot ID:
- Q14703
- Entry Name:
- MBTP1_HUMAN
- Status:
- reviewed
- Protein Names:
- Membrane-bound transcription factor site-1 protease (EC 3.4.21.112) (Endopeptidase S1P) (Subtilisin/kexin-isozyme 1) (SKI-1)
- Gene Names:
- MBTPS1 KIAA0091 S1P SKI1
- Gene Names Primary:
- MBTPS1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 1052
- Sequence:
- MKLVNIWLLLLVVLLCGKKHLGDRLEKKSFEKAPCPGCSHLTLKVEFSSTVVEYEYIVAFNGYFTAKARNSFISSALKSSEVDNWRIIPRNNPSSDYPSDFEVIQIKEKQKAGLLTLEDHPNIKRVTPQRKVFRSLKYAESDPTVPCNETRWSQKWQSSRPLRRASLSLGSGFWHATGRHSSRRLLRAIPRQVAQTLQADVLWQMGYTGANVRVAVFDTGLSEKHPHFKNVKERTNWTNERTLDDGLGHGTFVAGVIASMRECQGFAPDAELHIFRVFTNNQVSYTSWFLDAFNYAILKKIDVLNLSIGGPDFMDHPFVDKVWELTANNVIMVSAIGNDGPLYGTLNNPADQMDVIGVGGIDFEDNIARFSSRGMTTWELPGGYGRMKPDIVTYGAGVRGSGVKGGCRALSGTSVASPVVAGAVTLLVSTVQKRELVNPASMKQALIASARRLPGVNMFEQGHGKLDLLRAYQILNSYKPQASLSPSYIDLTECPYMWPYCSQPIYYGGMPTVVNVTILNGMGVTGRIVDKPDWQPYLPQNGDNIEVAFSYSSVLWPWSGYLAISISVTKKAASWEGIAQGHVMITVASPAETESKNGAEQTSTVKLPIKVKIIPTPPRSKRVLWDQYHNLRYPPGYFPRDNLRMKNDPLDWNGDHIHTNFRDMYQHLRSMGYFVEVLGAPFTCFDASQYGTLLMVDSEEEYFPEEIAKLRRDVDNGLSLVIFSDWYNTSVMRKVKFYDENTRQWWMPDTGGANIPALNELLSVWNMGFSDGLYEGEFTLANHDMYYASGCSIAKFPEDGVVITQTFKDQGLEVLKQETAVVENVPILGLYQIPAEGGGRIVLYGDSNCLDDSHRQKDCFWLLDALLQYTSYGVTPPSLSHSGNRQRPPSGAGSVTPERMEGNHLHRYSKVLEAHLGDPKPRPLPACPRLSWAKPQPLNETAPSNLWKHQKLLSIDLDKVVLPNFRSNRPQVRPLSPGESGAWDIPGGIMPGRYNQEVGQTIPVFAFLGAMVVLAFFVVQINKAKSRPKRRKPRVKRPQLMQQVHPPKTPSV
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Endoplasmic reticulum membrane
Function
- 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.
- Catalytic Activity:
- Processes precursors containing basic and hydrophobic/aliphatic residues at P4 and P2, respectively, with a relatively relaxed acceptance of amino acids at P1 and P3.
- Cofactor:
- COFACTOR: Name=Ca(2+); Xref=ChEBI:CHEBI:29108;
- Enzyme Regulation:
- ENZYME REGULATION: Inhibited by divalent copper and zinc ions, but not by nickel or cobalt. Inhibited by its prosegment, but not smaller fragments thereof.
- Active Site:
- ACT_SITE 218 218 Charge relay system.
- Gene Ontology Go:
- endoplasmic reticulum lumen
endoplasmic reticulum membrane
Golgi membrane
Golgi stack
integral component of membrane
serine-type endopeptidase activity
ATF6-mediated unfolded protein response
cellular protein metabolic process
cholesterol metabolic process
endoplasmic reticulum unfolded protein response
lysosome organization
membrane protein intracellular domain proteolysis
proteolysis
regulation of transcription factor import into nucleus
response to endoplasmic reticulum stress
small molecule metabolic process - Gene Ontology Biological Process:
- ATF6-mediated unfolded protein response
cellular protein metabolic process
cholesterol metabolic process
endoplasmic reticulum unfolded protein response
lysosome organization
membrane protein intracellular domain proteolysis
proteolysis
regulation of transcription factor import into nucleus
response to endoplasmic reticulum stress
small molecule metabolic process - Gene Ontology Molecular Function:
- serine-type endopeptidase activity
- Gene Ontology Cellular Component:
- endoplasmic reticulum lumen
endoplasmic reticulum membrane
Golgi membrane
Golgi stack
integral component of membrane - Keywords:
- Autocatalytic cleavage
Calcium
Cholesterol metabolism
Complete proteome
Direct protein sequencing
Endoplasmic reticulum
Glycoprotein
Golgi apparatus
Hydrolase
Lipid metabolism
Membrane
Phosphoprotein
Polymorphism
Protease
Reference proteome
Serine protease
Signal
Steroid metabolism
Sterol metabolism
Transmembrane
Transmembrane helix
Zymogen