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706 related items for PubMed ID: 16934036

  • 1. Hydrophilic iminosugar active-site-specific chaperones increase residual glucocerebrosidase activity in fibroblasts from Gaucher patients.
    Chang HH, Asano N, Ishii S, Ichikawa Y, Fan JQ.
    FEBS J; 2006 Sep; 273(17):4082-92. PubMed ID: 16934036
    [Abstract] [Full Text] [Related]

  • 2. Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.
    Luan Z, Higaki K, Aguilar-Moncayo M, Ninomiya H, Ohno K, García-Moreno MI, Ortiz Mellet C, García Fernández JM, Suzuki Y.
    Chembiochem; 2009 Nov 23; 10(17):2780-92. PubMed ID: 19830760
    [Abstract] [Full Text] [Related]

  • 3. Promising results of the chaperone effect caused by imino sugars and aminocyclitol derivatives on mutant glucocerebrosidases causing Gaucher disease.
    Sánchez-Ollé G, Duque J, Egido-Gabás M, Casas J, Lluch M, Chabás A, Grinberg D, Vilageliu L.
    Blood Cells Mol Dis; 2009 Nov 23; 42(2):159-66. PubMed ID: 19167250
    [Abstract] [Full Text] [Related]

  • 4. Gaucher disease-associated glucocerebrosidases show mutation-dependent chemical chaperoning profiles.
    Sawkar AR, Adamski-Werner SL, Cheng WC, Wong CH, Beutler E, Zimmer KP, Kelly JW.
    Chem Biol; 2005 Nov 23; 12(11):1235-44. PubMed ID: 16298303
    [Abstract] [Full Text] [Related]

  • 5. Role of pH in determining the cell-type-specific residual activity of glucocerebrosidase in type 1 Gaucher disease.
    van Weely S, van den Berg M, Barranger JA, Sa Miranda MC, Tager JM, Aerts JM.
    J Clin Invest; 1993 Mar 23; 91(3):1167-75. PubMed ID: 8450045
    [Abstract] [Full Text] [Related]

  • 6. Selective chaperone effect of aminocyclitol derivatives on G202R and other mutant glucocerebrosidases causing Gaucher disease.
    Serra-Vinardell J, Díaz L, Gutiérrez-de Terán H, Sánchez-Ollé G, Bujons J, Michelakakis H, Mavridou I, Aerts JM, Delgado A, Grinberg D, Vilageliu L, Casas J.
    Int J Biochem Cell Biol; 2014 Sep 23; 54():245-54. PubMed ID: 25084554
    [Abstract] [Full Text] [Related]

  • 7. Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants.
    Sawkar AR, Schmitz M, Zimmer KP, Reczek D, Edmunds T, Balch WE, Kelly JW.
    ACS Chem Biol; 2006 May 23; 1(4):235-51. PubMed ID: 17163678
    [Abstract] [Full Text] [Related]

  • 8. Rapid modifications of N-substitution in iminosugars: development of new β-glucocerebrosidase inhibitors and pharmacological chaperones for Gaucher disease.
    Cheng WC, Weng CY, Yun WY, Chang SY, Lin YC, Tsai FJ, Huang FY, Chen YR.
    Bioorg Med Chem; 2013 Sep 01; 21(17):5021-8. PubMed ID: 23880081
    [Abstract] [Full Text] [Related]

  • 9. Glucocerebrosidase inhibitors for the treatment of Gaucher disease.
    Trapero A, Llebaria A.
    Future Med Chem; 2013 Apr 01; 5(5):573-90. PubMed ID: 23573974
    [Abstract] [Full Text] [Related]

  • 10. Alpha-1-C-octyl-1-deoxynojirimycin as a pharmacological chaperone for Gaucher disease.
    Yu L, Ikeda K, Kato A, Adachi I, Godin G, Compain P, Martin O, Asano N.
    Bioorg Med Chem; 2006 Dec 01; 14(23):7736-44. PubMed ID: 16919960
    [Abstract] [Full Text] [Related]

  • 11. The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.
    Steet RA, Chung S, Wustman B, Powe A, Do H, Kornfeld SA.
    Proc Natl Acad Sci U S A; 2006 Sep 12; 103(37):13813-8. PubMed ID: 16945909
    [Abstract] [Full Text] [Related]

  • 12. Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease.
    Schmitz M, Alfalah M, Aerts JM, Naim HY, Zimmer KP.
    Int J Biochem Cell Biol; 2005 Nov 12; 37(11):2310-20. PubMed ID: 15982918
    [Abstract] [Full Text] [Related]

  • 13. A Fluorescent sp2-iminosugar with pharmacological chaperone activity for gaucher disease: synthesis and intracellular distribution studies.
    Luan Z, Higaki K, Aguilar-Moncayo M, Li L, Ninomiya H, Nanba E, Ohno K, García-Moreno MI, Ortiz Mellet C, García Fernández JM, Suzuki Y.
    Chembiochem; 2010 Nov 22; 11(17):2453-64. PubMed ID: 21064079
    [Abstract] [Full Text] [Related]

  • 14. Miglustat (NB-DNJ) works as a chaperone for mutated acid beta-glucosidase in cells transfected with several Gaucher disease mutations.
    Alfonso P, Pampín S, Estrada J, Rodríguez-Rey JC, Giraldo P, Sancho J, Pocoví M.
    Blood Cells Mol Dis; 2005 Nov 22; 35(2):268-76. PubMed ID: 16039881
    [Abstract] [Full Text] [Related]

  • 15. Glucosylceramide mimics: highly potent GCase inhibitors and selective pharmacological chaperones for mutations associated with types 1 and 2 Gaucher disease.
    Schönemann W, Gallienne E, Ikeda-Obatake K, Asano N, Nakagawa S, Kato A, Adachi I, Górecki M, Frelek J, Martin OR.
    ChemMedChem; 2013 Nov 22; 8(11):1805-17. PubMed ID: 24115322
    [Abstract] [Full Text] [Related]

  • 16. ER retention and degradation as the molecular basis underlying Gaucher disease heterogeneity.
    Ron I, Horowitz M.
    Hum Mol Genet; 2005 Aug 15; 14(16):2387-98. PubMed ID: 16000318
    [Abstract] [Full Text] [Related]

  • 17. Gaucher disease: functional expression of the normal glucocerebrosidase and Gaucher T1366G and G1604A alleles in Baculovirus-transfected Spodoptera frugiperda cells.
    Choy FY, Wei C, Levin D.
    Am J Med Genet; 1996 Oct 28; 65(3):184-9. PubMed ID: 9240741
    [Abstract] [Full Text] [Related]

  • 18. Gaucher disease: comparative study of acid phosphatase and glucocerebrosidase in normal and type-1 Gaucher tissues.
    Choy FY.
    Am J Med Genet; 1985 Jul 28; 21(3):519-28. PubMed ID: 4025386
    [Abstract] [Full Text] [Related]

  • 19. Rational design and synthesis of highly potent pharmacological chaperones for treatment of N370S mutant Gaucher disease.
    Wang GN, Reinkensmeier G, Zhang SW, Zhou J, Zhang LR, Zhang LH, Butters TD, Ye XS.
    J Med Chem; 2009 May 28; 52(10):3146-9. PubMed ID: 19397268
    [Abstract] [Full Text] [Related]

  • 20. Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.
    Yu Z, Sawkar AR, Whalen LJ, Wong CH, Kelly JW.
    J Med Chem; 2007 Jan 11; 50(1):94-100. PubMed ID: 17201413
    [Abstract] [Full Text] [Related]

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