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Literature for peptidase M50.001: site 2 peptidase

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Literature Substrates

(Topics flags: M Mutation, K Knockout, I Inhibitor, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

    2023
  1. Lim,P.J., Marcionelli,G., Srikanthan,P., Ndarugendamwo,T., Pinner,J., Rohrbach,M. and Giunta,C.
    Perturbations in fatty acid metabolism and collagen production infer pathogenicity of a novel MBTPS2 variant in Osteogenesis imperfecta
    Front Endocrinol (Lausanne)14, 1195704-1195704. PubMed  Europe PubMed DOI
    2. 2021
  2. Caengprasath,N., Theerapanon,T., Porntaveetus,T. and Shotelersuk,V.
    MBTPS2, a membrane bound protease, underlying several distinct skin and bone disorders
    J Transl Med19, 114-114. PubMed  Europe PubMed DOI  V
  3. Danyukova,T., Schoneck,K. and Pohl,S.
    Site-1 and site-2 proteases: A team of two in regulated proteolysis
    Biochim Biophys Acta Mol Cell Res1869, 119138-119138. PubMed  Europe PubMed DOI  V
  4. Lim,P.J., Marfurt,S., Lindert,U., Opitz,L., Ndarugendamwo,T., Srikanthan,P., Poms,M., Hersberger,M., Langhans,C.D., Haas,D., Rohrbach,M. and Giunta,C.
    Omics Profiling of S2P Mutant Fibroblasts as a Mean to Unravel the Pathomechanism and Molecular Signatures of X-Linked MBTPS2 Osteogenesis Imperfecta
    Front Genet12, 662751-662751. PubMed  Europe PubMed DOI
  5. Strong,A., March,M.E., Cardinale,C.J., Kim,S.E., Merves,J., Whitworth,H., Raffini,L., Larosa,C., Copelovitch,L., Hou,C., Slater,D., Vaccaro,C., Watson,D., Zackai,E.H., Billheimer,J. and Hakonarson,H.
    A novel MBTPS2 variant associated with BRESHECK syndrome impairs sterol-regulated transcription and the endoplasmic reticulum stress response
    Am J Med Genet A PubMed  Europe PubMed DOI
  6. Xiao,J., Xiong,Y., Yang,L.T., Wang,J.Q., Zhou,Z.M., Dong,L.W., Shi,X.J., Zhao,X., Luo,J. and Song,B.L.
    POST1/C12ORF49 regulates the SREBP pathway by promoting site-1 protease maturation
    Protein Cell12, 279-296. PubMed  Europe PubMed DOI
    7. 2020
  7. Liu,Y., Xu,D., Wang,L., Du,W., Zhang,L. and Xiang,X.
    MBTPS2 exacerbates albuminuria in streptozotocin-induced type I diabetic nephropathy by promoting endoplasmic reticulum stress-mediated renal damage
    Arch Physiol Biochem1-8. PubMed  Europe PubMed DOI
    8. 2016
  8. Lindert,U., Cabral,W.A., Ausavarat,S., Tongkobpetch,S., Ludin,K., Barnes,A.M., Yeetong,P., Weis,M., Krabichler,B., Srichomthong,C., Makareeva,E.N., Janecke,A.R., Leikin,S., Rothlisberger,B., Rohrbach,M., Kennerknecht,I., Eyre,D.R., Suphapeetiporn,K., Giunta,C., Marini,J.C. and Shotelersuk,V.
    MBTPS2 mutations cause defective regulated intramembrane proteolysis in X-linked osteogenesis imperfecta
    Nat Commun7, 11920-11920. PubMed  Europe PubMed DOI  M
    9. 2015
  9. Guan,M., Su,L., Yuan,Y.C., Li,H. and Chow,W.A.
    Nelfinavir and nelfinavir analogs block site-2 protease cleavage to inhibit castration-resistant prostate cancer
    Sci Rep5, 9698-9698. PubMed  Europe PubMed DOI  I
  10. Zhou,S.F., Sun,L., Valdes,A.E., Engstrom,P., Song,Z.T., Lu,S.J. and Liu,J.X.
    Membrane-associated transcription factor peptidase, site-2 protease, antagonizes ABA signaling in Arabidopsis
    New Phytol208, 188-197. PubMed  Europe PubMed DOI
    11. 2013
  11. Bornholdt,D., Atkinson,T.P., Bouadjar,B., Catteau,B., Cox,H., De Silva,D., Fischer,J., Gunasekera,C.N., Hadj-Rabia,S., Happle,R., Holder-Espinasse,M., Kaminski,E., Konig,A., Megarbane,A., Megarbane,H., Neidel,U., Oeffner,F., Oji,V., Theos,A., Traupe,H., Vahlquist,A., van Bon,B.W., Virtanen,M. and Grzeschik,K.H.
    Genotype-phenotype correlations emerging from the identification of missense mutations in MBTPS2
    Hum Mutat34, 587-594. PubMed  Europe PubMed DOI  M
  12. Kroos,L. and Akiyama,Y.
    Biochemical and structural insights into intramembrane metalloprotease mechanisms
    Biochim Biophys Acta1828, 2873-2885. PubMed  Europe PubMed DOI  V
  13. Morohashi,Y. and Tomita,T.
    Protein trafficking and maturation regulate intramembrane proteolysis
    Biochim Biophys Acta1828, 2855-2861. PubMed  Europe PubMed DOI  V
  14. Rawson,R.B.
    The site-2 protease
    Biochim Biophys Acta1828, 2801-2807. PubMed  Europe PubMed DOI  V
  15. Urban,S.
    Mechanisms and cellular functions of intramembrane proteases
    Biochim Biophys Acta1828, 2797-2800. PubMed  Europe PubMed DOI  V
    16. 2012
  16. Denard,B., Lee,C. and Ye,J.
    Doxorubicin blocks proliferation of cancer cells through proteolytic activation of CREB3L1
    elife1, e00090-e00090. PubMed  Europe PubMed DOI
  17. Rawson,R.B.
    S2P protease
    [ISSN:978-0-12-407743-0]3, 1536-1541. DOI
    18. 2011
  18. Oeffner,F., Martinez,F., Schaffer,J., Salhi,A., Monfort,S., Oltra,S., Neidel,U., Bornholdt,D., van Bon,B., Konig,A., Happle,R. and Grzeschik,K.H.
    Intronic mutations affecting splicing of MBTPS2 cause ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome
    Exp Dermatol20, 447-449. PubMed  Europe PubMed DOI  M
  19. Ozdemir,C. and Rawson,R.B.
    Other ways to skin a cat: activating SREBP without scap
    Fly (Austin)5, 3-6. PubMed  Europe PubMed  M  V
    20. 2010
  20. Aten,E., Brasz,L.C., Bornholdt,D., Hooijkaas,I.B., Porteous,M.E., Sybert,V.P., Vermeer,M.H., Vossen,R.H., van der Wielen,M.J., Bakker,E., Breuning,M.H., Grzeschik,K.H., Oosterwijk,J.C. and den Dunnen,J.T.
    Keratosis follicularis spinulosa decalvans is caused by mutations in MBTPS2
    Hum Mutat31, 1125-1133. PubMed  Europe PubMed DOI  M
  21. Chen,G. and Zhang,X.
    New insights into S2P signaling cascades: regulation, variation, and conservation
    Protein Sci19, 2015-2030. PubMed  Europe PubMed DOI  V
  22. Eleveld-Trancikova,D., Sanecka,A., van Hout-Kuijer,M.A., Looman,M.W., Hendriks,I.A., Jansen,B.J. and Adema,G.J.
    DC-STAMP interacts with ER-resident transcription factor LUMAN which becomes activated during DC maturation
    Mol Immunol47, 1963-1973. PubMed  Europe PubMed DOI
    23. 2009
  23. Ha,Y.
    Structure and mechanism of intramembrane protease
    Semin Cell Dev Biol20, 240-250. PubMed  Europe PubMed DOI  V
  24. Oeffner,F., Fischer,G., Happle,R., Konig,A., Betz,R.C., Bornholdt,D., Neidel,U., Boente,M., Redler,S., Romero-Gomez,J., Salhi,A., Vera-Casano,A., Weirich,C. and Grzeschik,K.H.
    IFAP syndrome is caused by deficiency in MBTPS2, an intramembrane zinc metalloprotease essential for cholesterol homeostasis and ER stress response
    Am J Hum Genet84, 459-467. PubMed  Europe PubMed DOI  M
  25. Wolfe,M.S.
    Intramembrane-cleaving proteases
    J Biol Chem284, 13969-13973. PubMed  Europe PubMed DOI
    26. 2006
  26. Murakami,T., Kondo,S., Ogata,M., Kanemoto,S., Saito,A., Wanaka,A. and Imaizumi,K.
    Cleavage of the membrane-bound transcription factor OASIS in response to endoplasmic reticulum stress
    J Neurochem96, 1090-1100. PubMed  Europe PubMed DOI
  27. Nadanaka,S., Yoshida,H., Sato,R. and Mori,K.
    Analysis of ATF6 activation in site-2 protease-deficient Chinese hamster ovary cells
    Cell Struct Funct31, 109-116. PubMed  Europe PubMed DOI  K
    28. 2004
  28. Rawson,R.B.
    S2P protease
    [ISSN:0-12-079610-4]2, 985-988.  V
  29. [YEAR:6-8-2004]Shen,J. and Prywes,R.
    Dependence of site-2 protease cleavage of ATF6 on prior site-1 protease digestion is determined by the size of the luminal domain of ATF6
    J Biol Chem279, 43046-43051. PubMed  Europe PubMed DOI
    30. 2003
  30. Okada,T., Haze,K., Nadanaka,S., Yoshida,H., Seidah,N.G., Hirano,Y., Sato,R., Negishi,M. and Mori,K.
    A serine protease inhibitor prevents endoplasmic reticulum stress-induced cleavage but not transport of the membrane-bound transcription factor ATF6
    J Biol Chem278, 31024-31032. PubMed  Europe PubMed DOI  I
  31. Weihofen,A. and Martoglio,B.
    Intramembrane-cleaving proteases: controlled liberation of proteins and bioactive peptides
    Trends Cell Biol13, 71-78. PubMed  Europe PubMed DOI
    32. 2002
  32. [YEAR:15-1-2002]Goldstein,J.L., Rawson,R.B. and Brown,M.S.
    Mutant mammalian cells as tools to delineate the sterol regulatory element-binding protein pathway for feedback regulation of lipid synthesis
    Arch Biochem Biophys397, 139-148. PubMed  Europe PubMed DOI
  33. [YEAR:15-2-2002]Lee,K., Tirasophon,W., Shen,X., Michalak,M., Prywes,R., Okada,T., Yoshida,H., Mori,K. and Kaufman,R.J.
    IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response
    Genes Dev16, 452-466. PubMed  Europe PubMed DOI
    34. 2000
  34. [YEAR:9-5-2000]Ye,J., Dave,U.P., Grishin,N.V., Goldstein,J.L. and Brown,M.S.
    Asparagine-proline sequence within membrane-spanning segment of SREBP triggers intramembrane cleavage by site-2 protease
    Proc Natl Acad Sci U S A97, 5123-5128. PubMed  Europe PubMed
  35. Ye,J., Rawson,R.B., Komuro,R., Chen,X., Dave,U.P., Prywes,R., Brown,M.S. and Goldstein,J.L.
    ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs
    Mol Cell6, 1355-1364. PubMed  Europe PubMed DOI
    36. 1999
  36. [YEAR:28-9-1999]Brown,M.S. and Goldstein,J.L.
    A proteolytic pathway that controls the cholesterol content of membranes, cells, and blood
    Proc Natl Acad Sci U S A96, 11041-11048. PubMed  Europe PubMed DOI
  37. Lewis,A.P. and Thomas,P.J.
    A novel clan of zinc metallopeptidases with possible intramembrane cleavage properties
    Protein Sci8, 439-442. PubMed  Europe PubMed DOI
  38. [YEAR:21-12-1999]Rudner,D.Z., Fawcett,P. and Losick,R.
    A family of membrane-embedded metalloproteases involved in regulated proteolysis of membrane-associated transcription factors
    Proc Natl Acad Sci U S A96, 14765-14770. PubMed  Europe PubMed DOI
  39. [YEAR:30-7-1999]Zelenski,N.G., Rawson,R.B., Brown,M.S. and Goldstein,J.L.
    Membrane topology of S2P, a protein required for intramembranous cleavage of sterol regulatory element-binding proteins
    J Biol Chem274, 21973-21980. PubMed  Europe PubMed DOI
    40. 1998
  40. [YEAR:10-7-1998]Duncan,E.A., Dave,U.P., Sakai,J., Goldstein,J.L. and Brown,M.S.
    Second-site cleavage in sterol regulatory element-binding protein occurs at transmembrane junction as determined by cysteine panning
    J Biol Chem273, 17801-17809. PubMed  Europe PubMed DOI
  41. [YEAR:15-5-1998]Manni,M.E., Cescato,R. and Paganetti,P.A.
    Lack of beta-amyloid production in M19 cells deficient in site 2 processing of the sterol regulatory element binding proteins
    FEBS Lett427, 367-370. PubMed  Europe PubMed DOI
  42. [YEAR:19-6-1998]Ross,S.L., Martin,F., Simonet,L., Jacobsen,F., Deshpande,R., Vassar,R., Bennett,B., Luo,Y., Wooden,S., Hu,S., Citron,M. and Burgess,T.L.
    Amyloid precursor protein processing in sterol regulatory element-binding protein site 2 protease-deficient Chinese hamster ovary cells
    J Biol Chem273, 15309-15312. PubMed  Europe PubMed DOI
  43. [YEAR:30-3-1998]Tomita,T., Chang,T.Y., Kodama,T. and Iwatsubo,T.
    BetaAPP gamma-secretase and SREBP site 2 protease are two different enzymes
    Neuroreport9, 911-913. PubMed  Europe PubMed
    44. 1997
  44. Brown,M.S. and Goldstein,J.L.
    The SREBP pathway: Regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor
    Cell89, 331-340. PubMed  Europe PubMed DOI
  45. Duncan,E.A., Brown,M.S., Goldstein,J.L. and Sakai,J.
    Cleavage site for sterol-regulated protease localized to a Leu-Ser bond in the lumenal loop of sterol regulatory element-binding protein-2
    J Biol Chem272, 12778-12785. PubMed  Europe PubMed DOI
  46. Rawson,R.B., Zelenski,N.G., Nijhawan,D., Ye,J., Sakai,J., Hasan,M.T., Chang,T.Y., Brown,M.S. and Goldstein,J.L.
    Complementation cloning of S2P, a gene encoding a putative metalloprotease required for intramembrane cleavage of SREBPs
    Mol Cell1, 47-57. PubMed  Europe PubMed
    47. 1996
  47. [YEAR:28-6-1996]Sakai,J., Duncan,E.A., Rawson,R.B., Hua,X., Brown,M.S. and Goldstein,J.L.
    Sterol-regulated release of SREBP-2 from cell membranes requires two sequential cleavages, one within a transmembrane segment
    Cell85, 1037-1046. PubMed  Europe PubMed DOI

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