ENZYME entry: EC

Accepted Name
Site-1 protease.
Alternative Name(s)
Mammalian subtilisin/kexin isozyme 1.
Membrane-bound transcription factor site-1 protease.
Proprotein convertase SKI-1.
Proprotein convertase SKI-1/S1PPS1.
S1P endopeptidase.
S1P protease.
Site-1 peptidase.
SREBP proteinase.
SREBP S1 protease.
SREBP-1 proteinase.
SREBP-2 proteinase.
Sterol regulatory element-binding protein proteinase.
Sterol regulatory element-binding protein site 1 protease.
Sterol-regulated luminal protease.
Subtilase SKI-1.
Subtilase SKI-1/S1P.
Subtilisin/kexin isozyme 1.
Reaction catalysed
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
  • Cleaves sterol regulatory element-binding proteins (SREBPs) and thereby initiates a process by which the active fragments of the SREBPs translocate to the nucleus and activate genes controlling the synthesis and uptake of cholesterol and unsaturated fatty acids into the bloodstream.
  • The enzyme also processes pro-brain-derived neurotrophic factor and undergoes autocatalytic activation in the endoplasmic reticulum through sequential cleavages.
  • The enzyme can also process the unfolded protein response stress factor ATF6 at an Arg-His-Lys-Lys-|- site, and the envelope glycoprotein of the highly infectious Lassa virus and Crimean Congo hemorrhagic fever virus at Arg-Arg-Lys-Lys-|-.
  • Belongs to peptidase family S8A.
PRIAM enzyme-specific profiles3.4.21.112
KEGG Ligand Database for Enzyme Nomenclature3.4.21.112
IUBMB Enzyme Nomenclature3.4.21.112
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