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212 related items for PubMed ID: 19106170

  • 1. 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; 328(3):723-31. PubMed ID: 19106170
    [Abstract] [Full Text] [Related]

  • 2. Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor.
    Fan JQ, Ishii S, Asano N, Suzuki Y.
    Nat Med; 1999 Jan; 5(1):112-5. PubMed ID: 9883849
    [Abstract] [Full Text] [Related]

  • 3. Pharmacological chaperone therapy by active-site-specific chaperones in Fabry disease: in vitro and preclinical studies.
    Germain DP, Fan JQ.
    Int J Clin Pharmacol Ther; 2009 Jan; 47 Suppl 1():S111-7. PubMed ID: 20040321
    [Abstract] [Full Text] [Related]

  • 4. 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]

  • 5. 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]

  • 6. 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]

  • 7. 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 01; 32(3):424-40. PubMed ID: 19387866
    [Abstract] [Full Text] [Related]

  • 8. Active-site-specific chaperone therapy for Fabry disease. Yin and Yang of enzyme inhibitors.
    Fan JQ, Ishii S.
    FEBS J; 2007 Oct 01; 274(19):4962-71. PubMed ID: 17894781
    [Abstract] [Full Text] [Related]

  • 9. 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]

  • 10. [Fabry's disease (alpha-galactosidase-A deficiency): recent therapeutic innovations].
    Germain DP.
    J Soc Biol; 2002 Apr 01; 196(2):183-90. PubMed ID: 12360747
    [Abstract] [Full Text] [Related]

  • 11. Enzyme therapy XVII: metabolic and immunologic evaluation of alpha- galactosidase A replacement in Fabry disease.
    Desnick RJ, Dean KJ, Grabowski GA, Bishop DF, Sweeley CC.
    Birth Defects Orig Artic Ser; 1980 Apr 01; 16(1):393-413. PubMed ID: 6256019
    [Abstract] [Full Text] [Related]

  • 12. N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis.
    Kasperzyk JL, El-Abbadi MM, Hauser EC, D'Azzo A, Platt FM, Seyfried TN.
    J Neurochem; 2004 May 01; 89(3):645-53. PubMed ID: 15086521
    [Abstract] [Full Text] [Related]

  • 13. A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder.
    Yam GH, Zuber C, Roth J.
    FASEB J; 2005 Jan 01; 19(1):12-8. PubMed ID: 15629890
    [Abstract] [Full Text] [Related]

  • 14. Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells.
    Takenaka T, Murray GJ, Qin G, Quirk JM, Ohshima T, Qasba P, Clark K, Kulkarni AB, Brady RO, Medin JA.
    Proc Natl Acad Sci U S A; 2000 Jun 20; 97(13):7515-20. PubMed ID: 10840053
    [Abstract] [Full Text] [Related]

  • 15. Partial correction of the alpha-galactosidase A deficiency and reduction of glycolipid storage in Fabry mice using synthetic vectors.
    Przybylska M, Wu IH, Zhao H, Ziegler RJ, Tousignant JD, Desnick RJ, Scheule RK, Cheng SH, Yew NS.
    J Gene Med; 2004 Jan 20; 6(1):85-92. PubMed ID: 14716680
    [Abstract] [Full Text] [Related]

  • 16. Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice.
    Jung SC, Han IP, Limaye A, Xu R, Gelderman MP, Zerfas P, Tirumalai K, Murray GJ, During MJ, Brady RO, Qasba P.
    Proc Natl Acad Sci U S A; 2001 Feb 27; 98(5):2676-81. PubMed ID: 11226298
    [Abstract] [Full Text] [Related]

  • 17. 4-Phenylbutyrate rescues trafficking incompetent mutant alpha-galactosidase A without restoring its functionality.
    Yam GH, Roth J, Zuber C.
    Biochem Biophys Res Commun; 2007 Aug 24; 360(2):375-80. PubMed ID: 17592721
    [Abstract] [Full Text] [Related]

  • 18. 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 Aug 24; 114():341-407. PubMed ID: 30635085
    [Abstract] [Full Text] [Related]

  • 19. Assessment of Gene Variant Amenability for Pharmacological Chaperone Therapy with 1-Deoxygalactonojirimycin in Fabry Disease.
    Lukas J, Cimmaruta C, Liguori L, Pantoom S, Iwanov K, Petters J, Hund C, Bunschkowski M, Hermann A, Cubellis MV, Rolfs A.
    Int J Mol Sci; 2020 Jan 31; 21(3):. PubMed ID: 32023956
    [Abstract] [Full Text] [Related]

  • 20. 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 31; 35(3):513-20. PubMed ID: 22187137
    [Abstract] [Full Text] [Related]

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