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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.
Gene Name: | membrane-bound transcription factor peptidase, site 1 |
Family/Subfamily: | Exopeptidase , Serine S8 |
Synonyms: | MBTPS1, Endopeptidase S1P, PCSK8, KIAA0091, Site-1 protease, Subtilisin/kexin isozyme-1, S1P, SKI-1, SKI1, Subtilisin/kexin-isozyme 1 |
Target Sequences: | NM_003791 NP_003782.1 Q14703 |
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