Gene/Proteome Database (LMPD)
LMPD ID
LMP001065
Gene ID
Species
Mus musculus (Mouse)
Gene Name
membrane-bound transcription factor peptidase, site 1
Gene Symbol
Synonyms
0610038M03Rik; AV003995; S1P; SKI-1; Ski1; mKIAA0091
Alternate Names
membrane-bound transcription factor site-1 protease; site-1 protease; S1P endopeptidase; endopeptidase S1P; subtilisin/kexin isozyme 1; subtilisin/kexin isozyme-1; subtilisin/kexin isozyme SKI-1; sterol-regulated luminal protease; membrane-bound transcription factor protease, site 1
Chromosome
8
Map Location
8 E1|8
EC Number
3.4.21.112
Proteins
| membrane-bound transcription factor site-1 protease precursor | |
|---|---|
| Refseq ID | NP_062683 |
| Protein GI | 268836117 |
| UniProt ID | Q9WTZ2 |
| mRNA ID | NM_019709 |
| Length | 1052 |
| RefSeq Status | VALIDATED |
| MKLVSTWLLVLVVLLCGKRHLGDRLGTRALEKAPCPSCSHLTLKVEFSSTVVEYEYIVAFNGYFTAKARNSFISSALKSSEVENWRIIPRNNPSSDYPSDFEVIQIKEKQKAGLLTLEDHPNIKRVTPQRKVFRSLKFAESNPIVPCNETRWSQKWQSSRPLKRASLSLGSGFWHATGRHSSRRLLRAIPRQVAQTLQADVLWQMGYTGANVRVAVFDTGLSEKHPHFKNVKERTNWTNERTLDDGLGHGTFVAGVIASMRECQGFAPDAELHIFRVFTNNQVSYTSWFLDAFNYAILKKMDVLNLSIGGPDFMDHPFVDKVWELTANNVIMVSAIGNDGPLYGTLNNPADQMDVIGVGGIDFEDNIARFSSRGMTTWELPGGYGRVKPDIVTYGAGVRGSGVKGGCRALSGTSVASPVVAGAVTLLVSTVQKRELVNPASVKQALIASARRLPGVNMFEQGHGKLDLLRAYQILSSYKPQASLSPSYIDLTECPYMWPYCSQPIYYGGMPTIVNVTILNGMGVTGRIVDKPEWRPYLPQNGDNIEVAFSYSSVLWPWSGYLAISISVTKKAASWEGIAQGHIMITVASPAETELHSGAEHTSTVKLPIKVKIIPTPPRSKRVLWDQYHNLRYPPGYFPRDNLRMKNDPLDWNGDHVHTNFRDMYQHLRSMGYFVEVLGAPFTCFDATQYGTLLLVDSEEEYFPEEIAKLRRDVDNGLSLVIFSDWYNTSVMRKVKFYDENTRQWWMPDTGGANIPALNELLSVWNMGFSDGLYEGEFVLANHDMYYASGCSIAKFPEDGVVITQTFKDQGLEVLKQETAVVENVPILGLYQIPSEGGGRIVLYGDSNCLDDSHRQKDCFWLLDALLQYTSYGVTPPSLSHSGNRQRPPSGAGLAPPERMEGNHLHRYSKVLEAHLGDPKPRPLPACPHLSWAKPQPLNETAPSNLWKHQKLLSIDLDKVVLPNFRSNRPQVRPLSPGESGAWDIPGGIMPGRYNQEVGQTIPVFAFLGAMVALAFFVVQISKAKSRPKRRRPRAKRPQLAQQAHPARTPSV | |
| sig_peptide: 1..17 inference: non-experimental evidence, no additional details recorded note: Potential; propagated from UniProtKB/Swiss-Prot (Q9WTZ2.1) calculated_mol_wt: 1887 peptide sequence: MKLVSTWLLVLVVLLCG mat_peptide: 187..1052 product: Membrane-bound transcription factor site-1 protease experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9WTZ2.1) calculated_mol_wt: 96274 peptide sequence: RAIPRQVAQTLQADVLWQMGYTGANVRVAVFDTGLSEKHPHFKNVKERTNWTNERTLDDGLGHGTFVAGVIASMRECQGFAPDAELHIFRVFTNNQVSYTSWFLDAFNYAILKKMDVLNLSIGGPDFMDHPFVDKVWELTANNVIMVSAIGNDGPLYGTLNNPADQMDVIGVGGIDFEDNIARFSSRGMTTWELPGGYGRVKPDIVTYGAGVRGSGVKGGCRALSGTSVASPVVAGAVTLLVSTVQKRELVNPASVKQALIASARRLPGVNMFEQGHGKLDLLRAYQILSSYKPQASLSPSYIDLTECPYMWPYCSQPIYYGGMPTIVNVTILNGMGVTGRIVDKPEWRPYLPQNGDNIEVAFSYSSVLWPWSGYLAISISVTKKAASWEGIAQGHIMITVASPAETELHSGAEHTSTVKLPIKVKIIPTPPRSKRVLWDQYHNLRYPPGYFPRDNLRMKNDPLDWNGDHVHTNFRDMYQHLRSMGYFVEVLGAPFTCFDATQYGTLLLVDSEEEYFPEEIAKLRRDVDNGLSLVIFSDWYNTSVMRKVKFYDENTRQWWMPDTGGANIPALNELLSVWNMGFSDGLYEGEFVLANHDMYYASGCSIAKFPEDGVVITQTFKDQGLEVLKQETAVVENVPILGLYQIPSEGGGRIVLYGDSNCLDDSHRQKDCFWLLDALLQYTSYGVTPPSLSHSGNRQRPPSGAGLAPPERMEGNHLHRYSKVLEAHLGDPKPRPLPACPHLSWAKPQPLNETAPSNLWKHQKLLSIDLDKVVLPNFRSNRPQVRPLSPGESGAWDIPGGIMPGRYNQEVGQTIPVFAFLGAMVALAFFVVQISKAKSRPKRRRPRAKRPQLAQQAHPARTPSV sig_peptide: 1..17 inference: non-experimental evidence, no additional details recorded note: Potential; propagated from UniProtKB/Swiss-Prot (Q9WTZ2.1) calculated_mol_wt: 1887 peptide sequence: MKLVSTWLLVLVVLLCG mat_peptide: 187..1052 product: Membrane-bound transcription factor site-1 protease experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9WTZ2.1) calculated_mol_wt: 96274 peptide sequence: RAIPRQVAQTLQADVLWQMGYTGANVRVAVFDTGLSEKHPHFKNVKERTNWTNERTLDDGLGHGTFVAGVIASMRECQGFAPDAELHIFRVFTNNQVSYTSWFLDAFNYAILKKMDVLNLSIGGPDFMDHPFVDKVWELTANNVIMVSAIGNDGPLYGTLNNPADQMDVIGVGGIDFEDNIARFSSRGMTTWELPGGYGRVKPDIVTYGAGVRGSGVKGGCRALSGTSVASPVVAGAVTLLVSTVQKRELVNPASVKQALIASARRLPGVNMFEQGHGKLDLLRAYQILSSYKPQASLSPSYIDLTECPYMWPYCSQPIYYGGMPTIVNVTILNGMGVTGRIVDKPEWRPYLPQNGDNIEVAFSYSSVLWPWSGYLAISISVTKKAASWEGIAQGHIMITVASPAETELHSGAEHTSTVKLPIKVKIIPTPPRSKRVLWDQYHNLRYPPGYFPRDNLRMKNDPLDWNGDHVHTNFRDMYQHLRSMGYFVEVLGAPFTCFDATQYGTLLLVDSEEEYFPEEIAKLRRDVDNGLSLVIFSDWYNTSVMRKVKFYDENTRQWWMPDTGGANIPALNELLSVWNMGFSDGLYEGEFVLANHDMYYASGCSIAKFPEDGVVITQTFKDQGLEVLKQETAVVENVPILGLYQIPSEGGGRIVLYGDSNCLDDSHRQKDCFWLLDALLQYTSYGVTPPSLSHSGNRQRPPSGAGLAPPERMEGNHLHRYSKVLEAHLGDPKPRPLPACPHLSWAKPQPLNETAPSNLWKHQKLLSIDLDKVVLPNFRSNRPQVRPLSPGESGAWDIPGGIMPGRYNQEVGQTIPVFAFLGAMVALAFFVVQISKAKSRPKRRRPRAKRPQLAQQAHPARTPSV | |
| membrane-bound transcription factor site-1 protease precursor | |
|---|---|
| Refseq ID | NP_001161382 |
| Protein GI | 268836232 |
| UniProt ID | Q9WTZ2 |
| mRNA ID | NM_001167910 |
| Length | 1052 |
| RefSeq Status | VALIDATED |
| Protein sequence is identical to GI:268836117 (mRNA isoform) | |
Gene Information
Entrez Gene ID
Gene Name
membrane-bound transcription factor peptidase, site 1
Gene Symbol
Species
Mus musculus
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005795 | IDA:MGI | C | Golgi stack |
| GO:0005788 | TAS:Reactome | C | endoplasmic reticulum lumen |
| GO:0016021 | IEA:UniProtKB-KW | C | integral component of membrane |
| GO:0004175 | IDA:MGI | F | endopeptidase activity |
| GO:0004252 | IDA:MGI | F | serine-type endopeptidase activity |
| GO:0008203 | IEA:UniProtKB-KW | P | cholesterol metabolic process |
| GO:0006629 | IMP:MGI | P | lipid metabolic process |
| GO:0007040 | ISS:UniProtKB | P | lysosome organization |
| GO:0006508 | ISS:UniProtKB | P | proteolysis |
| GO:0042990 | IDA:MGI | P | regulation of transcription factor import into nucleus |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| ko04141 | Protein processing in endoplasmic reticulum |
| mmu04141 | Protein processing in endoplasmic reticulum |
REACTOME Pathway Links
Domain Information
UniProt Annotations
Entry Information
Gene Name
membrane-bound transcription factor peptidase, site 1
Protein Entry
MBTP1_MOUSE
UniProt ID
Species
Mouse
Comments
| Comment Type | Description |
|---|---|
| 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 | Name=Ca(2+); Xref=ChEBI:CHEBI:29108; Evidence={ECO:0000250}; |
| Enzyme Regulation | Inhibited by divalent copper and zinc ions, but not by nickel or cobalt. Inhibited by its prosegment, but not smaller fragments thereof (By similarity). {ECO:0000250}. |
| 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 (By similarity). {ECO:0000250}. |
| Ptm | The 148 kDa zymogen is processed progressively into two membrane-bound 120 and 106 kDa forms in the endoplasmic reticulum, and late into a secreted 98 kDa form. The propeptide is autocatalytically removed through an intramolecular cleavage after Leu-186. Further cleavage generates 14, 10, and 8 kDa intermediates (By similarity). {ECO:0000250}. |
| Similarity | Belongs to the peptidase S8 family. {ECO:0000305}. |
| Similarity | Contains 1 peptidase S8 domain. {ECO:0000305}. |
| Subcellular Location | Endoplasmic reticulum membrane {ECO:0000250}; Single-pass type I membrane protein {ECO:0000250}. Golgi apparatus membrane {ECO:0000250}; Single-pass type I membrane protein {ECO:0000250}. Note=May sort to other organelles, including lysosomal and/or endosomal compartments. {ECO:0000250}. |
Identical and Related Proteins
Unique RefSeq proteins for LMP001065 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 268836117 | RefSeq | NP_062683 | 1052 | membrane-bound transcription factor site-1 protease precursor |
Identical Sequences to LMP001065 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:268836117 | DBBJ | BAC26263.1 | 1052 | unnamed protein product [Mus musculus] |
| GI:268836117 | GenBank | AAH54837.1 | 1052 | Membrane-bound transcription factor peptidase, site 1 [Mus musculus] |
| GI:268836117 | GenBank | EDL11600.1 | 1052 | membrane-bound transcription factor peptidase, site 1, isoform CRA_a [Mus musculus] |
| GI:268836117 | GenBank | ABT02355.1 | 1052 | Sequence 4 from patent US 7211424 |
| GI:268836117 | RefSeq | NP_001161382.1 | 1052 | membrane-bound transcription factor site-1 protease precursor [Mus musculus] |
| GI:268836117 | SwissProt | Q9WTZ2.1 | 1052 | RecName: Full=Membrane-bound transcription factor site-1 protease; AltName: Full=Endopeptidase S1P; AltName: Full=Sterol-regulated luminal protease; AltName: Full=Subtilisin/kexin isozyme 1; Short=SKI-1; Flags: Precursor [Mus musculus] |
Related Sequences to LMP001065 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:268836117 | DBBJ | BAC65484.1 | 1055 | mKIAA0091 protein, partial [Mus musculus] |
| GI:268836117 | EMBL | CAC08993.1 | 1052 | unnamed protein product [Rattus sp.] |
| GI:268836117 | GenBank | AAH57198.1 | 1052 | Membrane-bound transcription factor peptidase, site 1 [Mus musculus] |
| GI:268836117 | GenBank | ABK51402.1 | 1052 | membrane-bound transcription factor peptidase site 1 mutant [Mus musculus] |
| GI:268836117 | GenBank | EDL11601.1 | 1058 | membrane-bound transcription factor peptidase, site 1, isoform CRA_b, partial [Mus musculus] |
| GI:268836117 | GenBank | ABT02354.1 | 1052 | Sequence 2 from patent US 7211424 |