MBTPS1 (Q14703) - Overview - Molecular Target Synopsis
MBTPS1, Membrane-bound transcription factor site-1 protease
Enzyme Classification 18.104.22.168
Also Known as MBTP1_HUMAN, MBTPS1, KIAA0091, S1P, SKI1
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.
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UniProt: MBTPS1 is active in the following subcellular-locations: endoplasmic reticulum membrane, golgi apparatus membrane.
GO terms: MBTPS1 is active in the following subcellular-locations: endoplasmic reticulum lumen, endoplasmic reticulum membrane, Golgi apparatus, Golgi membrane, Golgi stack, integral component of membrane.