UniProt Protein Function: | MBTPS1: Catalyzes the first step in the proteolytic activation of the sterol regulatory element-binding proteins (SREBPs). Other known substrates are BDNF 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. Belongs to the peptidase S8 family. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Endoplasmic reticulum; EC 3.4.21.112; Protease Chromosomal Location of Human Ortholog: 16q24 Cellular Component: Golgi membrane; Golgi apparatus; endoplasmic reticulum membrane; Golgi stack; endoplasmic reticulum lumen; integral to membrane Molecular Function:serine-type endopeptidase activity Biological Process: cholesterol metabolic process; regulation of transcription factor import into nucleus; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; membrane protein intracellular domain proteolysis; lysosome organization and biogenesis; unfolded protein response; lipid metabolic process; proteolysis |
NCBI Summary: | This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to the cis/medial-Golgi where a second autocatalytic event takes place and the catalytic activity is acquired. It encodes a type 1 membrane bound protease which is ubiquitously expressed and regulates cholesterol or lipid homeostasis via cleavage of substrates at non-basic residues. Mutations in this gene may be associated with lysosomal dysfunction. [provided by RefSeq, Feb 2014] |
UniProt Code: | Q14703 |
NCBI GenInfo Identifier: | 17368466 |
NCBI Gene ID: | 8720 |
NCBI Accession: | Q14703.1 |
UniProt Secondary Accession: | Q14703,Q24JQ2, Q9UF67, A8K6V8, |
UniProt Related Accession: | Q14703 |
Molecular Weight: | |
NCBI Full Name: | Membrane-bound transcription factor site-1 protease |
NCBI Synonym Full Names: | membrane-bound transcription factor peptidase, site 1 |
NCBI Official Symbol: | MBTPS1 |
NCBI Official Synonym Symbols: | S1P; PCSK8; SKI-1 |
NCBI Protein Information: | membrane-bound transcription factor site-1 protease; endopeptidase S1P; subtilisin/kexin isozyme-1; proprotein convertase subtilisin/kexin type 8 |
UniProt Protein Name: | Membrane-bound transcription factor site-1 protease |
UniProt Synonym Protein Names: | Endopeptidase S1P; Subtilisin/kexin-isozyme 1; SKI-1 |
Protein Family: | Membrane-bound transcription factor site-1 protease |
UniProt Gene Name: | MBTPS1 |
UniProt Entry Name: | MBTP1_HUMAN |