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. \<br\>Perturbations in fatty acid metabolism and collagen production infer pathogenicity of a novel MBTPS2 variant in Osteogenesis imperfecta\<br\>Front Endocrinol (Lausanne) (2023) 14, 1195704. PubMed  Europe PubMed DOI  PMC  EPMC
  2. 2021
  3. Caengprasath,N., Theerapanon,T., Porntaveetus,T. and Shotelersuk,V. \<br\>MBTPS2, a membrane bound protease, underlying several distinct skin and bone disorders\<br\>J Transl Med (2021) 19, 114. PubMed  Europe PubMed DOI  PMC  EPMC  V
  4. Danyukova,T., Schoneck,K. and Pohl,S. \<br\>Site-1 and site-2 proteases: A team of two in regulated proteolysis\<br\>Biochim Biophys Acta (2021) 1869, 119138. PubMed  Europe PubMed DOI  V
  5. 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. \<br\>Omics Profiling of S2P Mutant Fibroblasts as a Mean to Unravel the Pathomechanism and Molecular Signatures of X-Linked MBTPS2 Osteogenesis Imperfecta\<br\>Front Genet (2021) 12, 662751. PubMed  Europe PubMed DOI  PMC  EPMC
  6. 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. \<br\>A novel MBTPS2 variant associated with BRESHECK syndrome impairs sterol-regulated transcription and the endoplasmic reticulum stress response\<br\>Am J Med Genet A (2021) PubMed  Europe PubMed DOI
  7. 2020
  8. Liu,Y., Xu,D., Wang,L., Du,W., Zhang,L. and Xiang,X. \<br\>MBTPS2 exacerbates albuminuria in streptozotocin-induced type I diabetic nephropathy by promoting endoplasmic reticulum stress-mediated renal damage\<br\>Arch Physiol Biochem (2020) 1-8. PubMed  Europe PubMed DOI
  9. 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. \<br\>POST1/C12ORF49 regulates the SREBP pathway by promoting site-1 protease maturation\<br\>Protein Cell (2020) PubMed  Europe PubMed DOI
  10. 2016
  11. 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. \<br\>MBTPS2 mutations cause defective regulated intramembrane proteolysis in X-linked osteogenesis imperfecta\<br\>Nat Commun (2016) 7, 11920. PubMed  Europe PubMed DOI  PMC  EPMC  M
  12. 2015
  13. Guan,M., Su,L., Yuan,Y.C., Li,H. and Chow,W.A. \<br\>Nelfinavir and nelfinavir analogs block site-2 protease cleavage to inhibit castration-resistant prostate cancer\<br\>Sci Rep (2015) 5, 9698. PubMed  Europe PubMed DOI  I
  14. Zhou,S.F., Sun,L., Valdes,A.E., Engstrom,P., Song,Z.T., Lu,S.J. and Liu,J.X. \<br\>Membrane-associated transcription factor peptidase, site-2 protease, antagonizes ABA signaling in Arabidopsis\<br\>New Phytol (2015) 208, 188-197. PubMed  Europe PubMed DOI
  15. 2013
  16. 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. \<br\>Genotype-phenotype correlations emerging from the identification of missense mutations in MBTPS2\<br\>Hum Mutat (2013) 34, 587-594. PubMed  Europe PubMed DOI  M
  17. Kroos,L. and Akiyama,Y. \<br\>Biochemical and structural insights into intramembrane metalloprotease mechanisms\<br\>Biochim Biophys Acta (2013) 1828, 2873-2885. PubMed  Europe PubMed DOI  PMC  EPMC  V
  18. Morohashi,Y. and Tomita,T. \<br\>Protein trafficking and maturation regulate intramembrane proteolysis\<br\>Biochim Biophys Acta (2013) 1828, 2855-2861. PubMed  Europe PubMed DOI  V
  19. Rawson,R.B. \<br\>The site-2 protease\<br\>Biochim Biophys Acta (2013) 1828, 2801-2807. PubMed  Europe PubMed DOI  V
  20. Urban,S. \<br\>Mechanisms and cellular functions of intramembrane proteases\<br\>Biochim Biophys Acta (2013) 1828, 2797-2800. PubMed  Europe PubMed DOI  V
  21. 2012
  22. Denard,B., Lee,C. and Ye,J. \<br\>Doxorubicin blocks proliferation of cancer cells through proteolytic activation of CREB3L1\<br\>elife (2012) 1, e00090. PubMed  Europe PubMed DOI  PMC  EPMC
  23. Rawson,R.B. \<br\>S2P protease\<br\>[ISSN:978-0-12-407743-0] (2012) 3, 1536-1541. DOI
  24. 2011
  25. 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. \<br\>Intronic mutations affecting splicing of MBTPS2 cause ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome\<br\>Exp Dermatol (2011) 20, 447-449. PubMed  Europe PubMed DOI  M
  26. Ozdemir,C. and Rawson,R.B. \<br\>Other ways to skin a cat: activating SREBP without scap\<br\>Fly (Austin) (2011) 5, 3-6. PubMed  Europe PubMed  M  V
  27. 2010
  28. 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. \<br\>Keratosis follicularis spinulosa decalvans is caused by mutations in MBTPS2\<br\>Hum Mutat (2010) 31, 1125-1133. PubMed  Europe PubMed DOI  M
  29. Chen,G. and Zhang,X. \<br\>New insights into S2P signaling cascades: regulation, variation, and conservation\<br\>Protein Sci (2010) 19, 2015-2030. PubMed  Europe PubMed DOI  V
  30. Eleveld-Trancikova,D., Sanecka,A., van Hout-Kuijer,M.A., Looman,M.W., Hendriks,I.A., Jansen,B.J. and Adema,G.J. \<br\>DC-STAMP interacts with ER-resident transcription factor LUMAN which becomes activated during DC maturation\<br\>Mol Immunol (2010) 47, 1963-1973. PubMed  Europe PubMed DOI
  31. 2009
  32. Ha,Y. \<br\>Structure and mechanism of intramembrane protease\<br\>Semin Cell Dev Biol (2009) 20, 240-250. PubMed  Europe PubMed DOI  PMC  EPMC  V
  33. 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. \<br\>IFAP syndrome is caused by deficiency in MBTPS2, an intramembrane zinc metalloprotease essential for cholesterol homeostasis and ER stress response\<br\>Am J Hum Genet (2009) 84, 459-467. PubMed  Europe PubMed DOI  PMC  EPMC  M
  34. Wolfe,M.S. \<br\>Intramembrane-cleaving proteases\<br\>J Biol Chem (2009) 284, 13969-13973. PubMed  Europe PubMed DOI  PMC  EPMC
  35. 2006
  36. Murakami,T., Kondo,S., Ogata,M., Kanemoto,S., Saito,A., Wanaka,A. and Imaizumi,K. \<br\>Cleavage of the membrane-bound transcription factor OASIS in response to endoplasmic reticulum stress\<br\>J Neurochem (2006) 96, 1090-1100. PubMed  Europe PubMed DOI
  37. Nadanaka,S., Yoshida,H., Sato,R. and Mori,K. \<br\>Analysis of ATF6 activation in site-2 protease-deficient Chinese hamster ovary cells\<br\>Cell Struct Funct (2006) 31, 109-116. PubMed  Europe PubMed DOI  K
  38. 2004
  39. Rawson,R.B. \<br\>S2P protease\<br\>[ISSN:0-12-079610-4] (2004) 2, 985-988.  V
  40. [YEAR:6-8-2004]Shen,J. and Prywes,R. \<br\>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\<br\>J Biol Chem (6-8-2004) 279, 43046-43051. PubMed  Europe PubMed DOI
  41. 2003
  42. Okada,T., Haze,K., Nadanaka,S., Yoshida,H., Seidah,N.G., Hirano,Y., Sato,R., Negishi,M. and Mori,K. \<br\>A serine protease inhibitor prevents endoplasmic reticulum stress-induced cleavage but not transport of the membrane-bound transcription factor ATF6\<br\>J Biol Chem (2003) 278, 31024-31032. PubMed  Europe PubMed DOI  I
  43. Weihofen,A. and Martoglio,B. \<br\>Intramembrane-cleaving proteases: controlled liberation of proteins and bioactive peptides\<br\>Trends Cell Biol (2003) 13, 71-78. PubMed  Europe PubMed DOI
  44. 2002
  45. [YEAR:15-1-2002]Goldstein,J.L., Rawson,R.B. and Brown,M.S. \<br\>Mutant mammalian cells as tools to delineate the sterol regulatory element-binding protein pathway for feedback regulation of lipid synthesis\<br\>Arch Biochem Biophys (15-1-2002) 397, 139-148. PubMed  Europe PubMed DOI
  46. [YEAR:15-2-2002]Lee,K., Tirasophon,W., Shen,X., Michalak,M., Prywes,R., Okada,T., Yoshida,H., Mori,K. and Kaufman,R.J. \<br\>IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response\<br\>Genes Dev (15-2-2002) 16, 452-466. PubMed  Europe PubMed DOI  PMC  EPMC
  47. 2000
  48. Ye,J., Rawson,R.B., Komuro,R., Chen,X., Dave,U.P., Prywes,R., Brown,M.S. and Goldstein,J.L. \<br\>ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs\<br\>Mol Cell (2000) 6, 1355-1364. PubMed  Europe PubMed DOI
  49. [YEAR:9-5-2000]Ye,J., Dave,U.P., Grishin,N.V., Goldstein,J.L. and Brown,M.S. \<br\>Asparagine-proline sequence within membrane-spanning segment of SREBP triggers intramembrane cleavage by site-2 protease\<br\>Proc Natl Acad Sci U S A (9-5-2000) 97, 5123-5128. PubMed  Europe PubMed  PMC  EPMC
  50. 1999
  51. [YEAR:28-9-1999]Brown,M.S. and Goldstein,J.L. \<br\>A proteolytic pathway that controls the cholesterol content of membranes, cells, and blood\<br\>Proc Natl Acad Sci U S A (28-9-1999) 96, 11041-11048. PubMed  Europe PubMed DOI  PMC  EPMC
  52. Lewis,A.P. and Thomas,P.J. \<br\>A novel clan of zinc metallopeptidases with possible intramembrane cleavage properties\<br\>Protein Sci (1999) 8, 439-442. PubMed  Europe PubMed DOI  PMC  EPMC
  53. [YEAR:21-12-1999]Rudner,D.Z., Fawcett,P. and Losick,R. \<br\>A family of membrane-embedded metalloproteases involved in regulated proteolysis of membrane-associated transcription factors\<br\>Proc Natl Acad Sci U S A (21-12-1999) 96, 14765-14770. PubMed  Europe PubMed DOI  PMC  EPMC
  54. [YEAR:30-7-1999]Zelenski,N.G., Rawson,R.B., Brown,M.S. and Goldstein,J.L. \<br\>Membrane topology of S2P, a protein required for intramembranous cleavage of sterol regulatory element-binding proteins\<br\>J Biol Chem (30-7-1999) 274, 21973-21980. PubMed  Europe PubMed DOI
  55. 1998
  56. [YEAR:10-7-1998]Duncan,E.A., Dave,U.P., Sakai,J., Goldstein,J.L. and Brown,M.S. \<br\>Second-site cleavage in sterol regulatory element-binding protein occurs at transmembrane junction as determined by cysteine panning\<br\>J Biol Chem (10-7-1998) 273, 17801-17809. PubMed  Europe PubMed DOI
  57. [YEAR:15-5-1998]Manni,M.E., Cescato,R. and Paganetti,P.A. \<br\>Lack of beta-amyloid production in M19 cells deficient in site 2 processing of the sterol regulatory element binding proteins\<br\>FEBS Lett (15-5-1998) 427, 367-370. PubMed  Europe PubMed DOI
  58. [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. \<br\>Amyloid precursor protein processing in sterol regulatory element-binding protein site 2 protease-deficient Chinese hamster ovary cells\<br\>J Biol Chem (19-6-1998) 273, 15309-15312. PubMed  Europe PubMed DOI
  59. [YEAR:30-3-1998]Tomita,T., Chang,T.Y., Kodama,T. and Iwatsubo,T. \<br\>BetaAPP gamma-secretase and SREBP site 2 protease are two different enzymes\<br\>Neuroreport (30-3-1998) 9, 911-913. PubMed  Europe PubMed
  60. 1997
  61. Brown,M.S. and Goldstein,J.L. \<br\>The SREBP pathway: Regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor\<br\>Cell (1997) 89, 331-340. PubMed  Europe PubMed DOI
  62. Duncan,E.A., Brown,M.S., Goldstein,J.L. and Sakai,J. \<br\>Cleavage site for sterol-regulated protease localized to a Leu-Ser bond in the lumenal loop of sterol regulatory element-binding protein-2\<br\>J Biol Chem (1997) 272, 12778-12785. PubMed  Europe PubMed DOI
  63. 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. \<br\>Complementation cloning of S2P, a gene encoding a putative metalloprotease required for intramembrane cleavage of SREBPs\<br\>Mol Cell (1997) 1, 47-57. PubMed  Europe PubMed
  64. 1996
  65. [YEAR:28-6-1996]Sakai,J., Duncan,E.A., Rawson,R.B., Hua,X., Brown,M.S. and Goldstein,J.L. \<br\>Sterol-regulated release of SREBP-2 from cell membranes requires two sequential cleavages, one within a transmembrane segment\<br\>Cell (28-6-1996) 85, 1037-1046. PubMed  Europe PubMed DOI