ENZYME

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A new class EC 7, Translocases, has been added to the EC list. It will be part of ENZYME from release 2018_10. Read more about EC 7 here.

ENZYME entry: EC 3.4.21.112

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.

SKI-1.

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

Comment(s)

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.

Cross-references

PROSITE

BRENDA

EC2PDB

ExplorEnz

PRIAM enzyme-specific profiles

3.4.21.112

KEGG Ligand Database for Enzyme Nomenclature