Membrane-Bound Transcription Factor Site-1 Protease (MBTPS1), Partial (MBTPS1) Human Recombinant Protein

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]

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.

Protein type: Endoplasmic reticulum; EC 3.4.21.112; Protease; Membrane protein, integral

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; unfolded protein response, activation of signaling protein activity; membrane protein intracellular domain proteolysis; cellular protein metabolic process; unfolded protein response; lysosome organization and biogenesis; lipid metabolic process; proteolysis