Fabry cardiomyopathy presenting as hypertrophic phenotype with left ventricular outflow tract obstruction: a case series. [PDF]
Front Cardiovasc MedHosyanto FF, Li Y, Luo S, Huang B.
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Beneficial bystander-enhanced cryptic splice rescue of cardiac-type Fabry GLA IVS4+919G>A by adenine base editing in patient fibroblasts. [PDF]
Gene TherChao HC +9 more
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Concurrent fabry disease and monoclonal gammopathy of renal significance: a case report. [PDF]
BMC NephrolLi XY, Chen QN, Fang JN, Zeng ZC, Pan Y.
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BMB Reports, 2009 Globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) are the proposed functional receptors for Shiga toxin (Stx). To elucidate the effect of Gb3 content on Stx-induced cytotoxicity in HeLa cells, we cloned HeLa cells and determined the correlation between glycolipids content and Stx-induced cytotoxicity. The 29 HeLa cell clone (HLCC) lines used
Satoshi Ishii
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Enzymatic synthesis of Gb3 and iGb3 ceramides [PDF]
Carbohydrate Research, 2010 Gb3 and iGb3 are physiologically important trihexosylceramides with a terminal alpha-d-Galp-(1-->4)-beta-d-Galp- and alpha-d-Galp-(1-->3)-beta-d-Galp sequence, respectively. In particular iGb3 is attracting considerable attention as it is believed to serve as a ligand for natural killer T cells. Whether or not iGb3 is present in humans and which enzyme
Norman J. Dovichi +2 more
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Synthesis of an Aminooxy Derivative of the Trisaccharide Globotriose Gb3 [PDF]
Journal of Carbohydrate Chemistry, 2014 The synthesis of α-aminooxy trisaccharide moiety [α-d-Gal-(1,4)-β-d-Gal-(1,4)-β-d-Glc-α-aminooxy], related to the cell surface globotriaosylceramide (Gb3) receptor of the B subunit of the AB5 Shiga toxin of Shigella dysenteriae, has been synthesized for the first time in 11 steps with a 15% overall isolated yield.
Peter R Andreana, Samir Ghosh
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Chemoenzymatic Syntheses of iGb3 and Gb3
Organic Letters, 2006Efficient chemoenzymatic syntheses of iGb3 and Gb3 have been developed. Isoglobotrihexose and globotrihexose were enzymatically synthesized by a three-enzyme system in both solid and solution phases. Then iGb3 and Gb3 were chemically synthesized by coupling of the corresponding trisaccharides with lipid.
Qingjia, Yao +4 more
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Transmembrane BAX Inhibitor Motif Containing (TMBIM) Family Proteins Perturbs a trans-Golgi Network Enzyme, Gb3 Synthase, and Reduces Gb3 Biosynthesis * [PDF]
Journal of Biological Chemistry, 2010 Globotriaosylceramide (Gb3) is a well known receptor for Shiga toxin (Stx), produced by enterohemorrhagic Escherichia coli and Shigella dysenteriae. The expression of Gb3 also affects several diseases, including cancer metastasis and Fabry disease, which prompted us to look for factors involved in its metabolism.
Kiyotaka Nishikawa +2 more
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Comparison of Total Lipid Composition in Gb3‐Positive and Gb3‐Deficient Burkitt's Lymphoma Cells
Journal of Liquid Chromatography & Related Technologies, 2005Abstract Thin-layer chromatography was used to compare the glycolipid, phospholipid, and neutral lipid compositions of the globotriaosylceramide (Gb3)‐positive Burkitt's lymphoma Daudi cell line and the Gb3‐deficient Daudi‐derived VT500 cell line. Glycolipid compositions were determined by co‐migration of lipids with glycosphingolipid standards and ...
Mark Maloney +3 more
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Total synthesis of globotriaosylceramide (Gb3) and lysoglobotriaosylceramide (lysoGb3)
Carbohydrate Research, 1990We have recently reported a highly efficient and stereocontrolled synthesis of globotriaosylceramide (Gb3, 1) in optically pure form. Key to our synthetic strategy was the implementation of the two-stage activation of thioglycosides for formation of the glycosidic bonds and the utilization of (2S, 3S, 4E)-2-azido-3-O-(tert-butyldimethylsilyl)-4 ...
K C, Nicolaou +2 more
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