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Journal Abstract Search

395 related items for PubMed ID: 19387866

  • 1. The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines.
    Benjamin ER, Flanagan JJ, Schilling A, Chang HH, Agarwal L, Katz E, Wu X, Pine C, Wustman B, Desnick RJ, Lockhart DJ, Valenzano KJ.
    J Inherit Metab Dis; 2009 Jun; 32(3):424-40. PubMed ID: 19387866
    [Abstract] [Full Text] [Related]

  • 2. A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease.
    Wu X, Katz E, Della Valle MC, Mascioli K, Flanagan JJ, Castelli JP, Schiffmann R, Boudes P, Lockhart DJ, Valenzano KJ, Benjamin ER.
    Hum Mutat; 2011 Aug; 32(8):965-77. PubMed ID: 21598360
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  • 3. Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin.
    Ishii S, Chang HH, Kawasaki K, Yasuda K, Wu HL, Garman SC, Fan JQ.
    Biochem J; 2007 Sep 01; 406(2):285-95. PubMed ID: 17555407
    [Abstract] [Full Text] [Related]

  • 4. Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variants.
    Yam GH, Bosshard N, Zuber C, Steinmann B, Roth J.
    Am J Physiol Cell Physiol; 2006 Apr 01; 290(4):C1076-82. PubMed ID: 16531566
    [Abstract] [Full Text] [Related]

  • 5. α-Galactosidase aggregation is a determinant of pharmacological chaperone efficacy on Fabry disease mutants.
    Siekierska A, De Baets G, Reumers J, Gallardo R, Rudyak S, Broersen K, Couceiro J, Van Durme J, Schymkowitz J, Rousseau F.
    J Biol Chem; 2012 Aug 17; 287(34):28386-97. PubMed ID: 22773828
    [Abstract] [Full Text] [Related]

  • 6. Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease.
    Ishii S, Yoshioka H, Mannen K, Kulkarni AB, Fan JQ.
    Biochim Biophys Acta; 2004 Nov 05; 1690(3):250-7. PubMed ID: 15511632
    [Abstract] [Full Text] [Related]

  • 7. The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
    Khanna R, Soska R, Lun Y, Feng J, Frascella M, Young B, Brignol N, Pellegrino L, Sitaraman SA, Desnick RJ, Benjamin ER, Lockhart DJ, Valenzano KJ.
    Mol Ther; 2010 Jan 05; 18(1):23-33. PubMed ID: 19773742
    [Abstract] [Full Text] [Related]

  • 8. Molecular basis of 1-deoxygalactonojirimycin arylthiourea binding to human α-galactosidase a: pharmacological chaperoning efficacy on Fabry disease mutants.
    Yu Y, Mena-Barragán T, Higaki K, Johnson JL, Drury JE, Lieberman RL, Nakasone N, Ninomiya H, Tsukimura T, Sakuraba H, Suzuki Y, Nanba E, Mellet CO, García Fernández JM, Ohno K.
    ACS Chem Biol; 2014 Jul 18; 9(7):1460-9. PubMed ID: 24783948
    [Abstract] [Full Text] [Related]

  • 9. Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease.
    Porto C, Pisani A, Rosa M, Acampora E, Avolio V, Tuzzi MR, Visciano B, Gagliardo C, Materazzi S, la Marca G, Andria G, Parenti G.
    J Inherit Metab Dis; 2012 May 18; 35(3):513-20. PubMed ID: 22187137
    [Abstract] [Full Text] [Related]

  • 10. Computational and modeling approaches to understand the impact of the Fabry's disease causing mutation (D92Y) on the interaction with pharmacological chaperone 1-deoxygalactonojirimycin (DGJ).
    Thirumal Kumar D, Judith E, Priyadharshini Christy J, Siva R, Tayubi IA, Chakraborty C, George Priya Doss C, Zayed H.
    Adv Protein Chem Struct Biol; 2019 May 18; 114():341-407. PubMed ID: 30635085
    [Abstract] [Full Text] [Related]

  • 11. Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone.
    Shin SH, Kluepfel-Stahl S, Cooney AM, Kaneski CR, Quirk JM, Schiffmann R, Brady RO, Murray GJ.
    Pharmacogenet Genomics; 2008 Sep 18; 18(9):773-80. PubMed ID: 18698230
    [Abstract] [Full Text] [Related]

  • 12. Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease.
    Ishii S, Chang HH, Yoshioka H, Shimada T, Mannen K, Higuchi Y, Taguchi A, Fan JQ.
    J Pharmacol Exp Ther; 2009 Mar 18; 328(3):723-31. PubMed ID: 19106170
    [Abstract] [Full Text] [Related]

  • 13. Effects of a chemical chaperone on genetic mutations in alpha-galactosidase A in Korean patients with Fabry disease.
    Park JY, Kim GH, Kim SS, Ko JM, Lee JJ, Yoo HW.
    Exp Mol Med; 2009 Jan 31; 41(1):1-7. PubMed ID: 19287194
    [Abstract] [Full Text] [Related]

  • 14. The molecular basis of pharmacological chaperoning in human α-galactosidase.
    Guce AI, Clark NE, Rogich JJ, Garman SC.
    Chem Biol; 2011 Dec 23; 18(12):1521-6. PubMed ID: 22195554
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  • 16. Pharmacological chaperone therapy for Fabry disease.
    Ishii S.
    Proc Jpn Acad Ser B Phys Biol Sci; 2012 Dec 23; 88(1):18-30. PubMed ID: 22241068
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