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Restricted accumulation of globotriaosylceramide in the hearts of atypical cases of Fabry's disease
Human Pathology, 1990Immunohistochemical and biochemical analyses of several tissues were performed in two unusual cases of Fabry's disease which showed accumulation of globotriaosylceramide (Gal alpha 1-4Gal beta 1-4 Glc-Cer, Gb3Cer) only in the hearts, but no clinical signs of the disease. Immunohistochemical study revealed that the hearts from our cases (cases no. 1 and
K, Ogawa +6 more
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Rapid Communications in Mass Spectrometry, 2005
Abstract Globotriaosylceramide is a neutral glycolipid containing the trihexoside Gal( α 1‐4)Gal( ß 1‐4)Glc( ß 1‐1′) covalently bound to N‐acylsphingosine.
Guenter, Fauler +7 more
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Abstract Globotriaosylceramide is a neutral glycolipid containing the trihexoside Gal( α 1‐4)Gal( ß 1‐4)Glc( ß 1‐1′) covalently bound to N‐acylsphingosine.
Guenter, Fauler +7 more
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Use of urinary globotriaosylceramide for fabry disease screening in Canada
Clinical Therapeutics, 20082008 S79 Clin Ther. 2008;30(Suppl C):S79–S80 © 2008 Excerpta Medica Inc. All rights reserved. 0149-2918/$32.00 Use of Urinary Globotriaosylceramide for Fabry Disease Screening in Canada Christiane Auray-Blais, LLM, PhD1; Denis Cyr, MSc1; Regen Drouin, MD, PhD1; Joe T.R. Clarke, MD, PhD1; and David S.
C AURAYBLAIS +4 more
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Chemistry and Physics of Lipids, 1990
Globotriaosylceramide, the natural substrate of alpha-galactosidase A (the enzyme deficient in Fabry's disease) was prepared from human kidney by repeated medium pressure chromatography on Lichroprep Si 60 (E. Merck) before and after peracetylation. The apparently homogeneous preparation migrating as a single band on HPTLC was analysed by fast atom ...
B, Röder +6 more
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Globotriaosylceramide, the natural substrate of alpha-galactosidase A (the enzyme deficient in Fabry's disease) was prepared from human kidney by repeated medium pressure chromatography on Lichroprep Si 60 (E. Merck) before and after peracetylation. The apparently homogeneous preparation migrating as a single band on HPTLC was analysed by fast atom ...
B, Röder +6 more
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Molecular Genetics and Metabolism, 2008
Fabry disease is a complex, multisystemic and clinically heterogeneous disease, in which the urinary excretion of globotriaosylceramide (Gb3), the principal substrate of the deficient enzyme, alpha-galactosidase A, is more prominent than the increased concentrations of the lipid in the plasma of affected hemizygotes and heterozygotes. We have developed
Auray-Blais, Christiane +11 more
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Fabry disease is a complex, multisystemic and clinically heterogeneous disease, in which the urinary excretion of globotriaosylceramide (Gb3), the principal substrate of the deficient enzyme, alpha-galactosidase A, is more prominent than the increased concentrations of the lipid in the plasma of affected hemizygotes and heterozygotes. We have developed
Auray-Blais, Christiane +11 more
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Rapid synthesis of oligosaccharide moieties of globotriaosylceramide using fluorous protective group
Tetrahedron Letters, 2003Abstract The use of the Bfp (bisfluorous chain type propanoyl) group as a fluorous protective group made it possible to rapidly synthesize galabiose and the Gb3 oligosaccharide derivatives by a simple fluorous–organic extraction purification. The fluorous oligosaccharide synthesis using the Bfp group is an excellent strategic alternative to solid ...
Tsuyoshi Miura, Toshiyuki Inazu
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Archives of Biochemistry and Biophysics, 1987
The activator protein for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide was purified from human kidney, brain, and urine. As far as they could be assayed, these three activities cochromatographed during all steps, indicating that they are due to the same protein.
A, Vogel +5 more
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The activator protein for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide was purified from human kidney, brain, and urine. As far as they could be assayed, these three activities cochromatographed during all steps, indicating that they are due to the same protein.
A, Vogel +5 more
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628: Cellular and tissue distribution of globotriaosylceramide in fabry disease
Journal of Clinical Neuroscience, 2007Hasan Askari +4 more
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The sub-cellular localization globotriaosylceramide in Fabry disease
Virchows Archiv, 2008openaire +1 more source