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Journal Abstract Search

244 related items for PubMed ID: 20961863

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  • 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; 18(1):23-33. PubMed ID: 19773742
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  • 12. Naked plasmid DNA-based alpha-galactosidase A gene transfer partially reduces systemic accumulation of globotriaosylceramide in Fabry mice.
    Nakamura G, Maruyama H, Ishii S, Shimotori M, Kameda S, Kono T, Miyazaki J, Kulkarni AB, Gejyo F.
    Mol Biotechnol; 2008 Feb; 38(2):109-19. PubMed ID: 18219591
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  • 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; 19(1):12-8. PubMed ID: 15629890
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  • 17. 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; 35(3):513-20. PubMed ID: 22187137
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  • 19. 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
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  • 20. Globotriaosylceramide induces lysosomal degradation of endothelial KCa3.1 in fabry disease.
    Choi S, Kim JA, Na HY, Cho SE, Park S, Jung SC, Suh SH.
    Arterioscler Thromb Vasc Biol; 2014 Jan 01; 34(1):81-9. PubMed ID: 24158513
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