Results 141 to 150 of about 3,830 (166)
Some of the next articles are maybe not open access.
Globotriaosylceramide inhibits iNKT‐cell activation in a CD1d‐dependent manner
European Journal of Immunology, 2015Globotriaosylceramide (Gb3) is a glycosphingolipid present in cellular membranes that progressively accumulates in Fabry disease. Invariant Natural Killer T (iNKT) cells are a population of lipid‐specific T cells that are phenotypically and functionally altered in Fabry disease.
Pereira, C. S. +4 more
openaire +4 more sources
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
openaire +2 more sources
Clinical and Experimental Ophthalmology, 2006
AbstractBackground: Fabry disease is an X‐linked disorder due to a deficiency of α‐galactosidase A and leads to the accumulation of globotriaosylceramide (Gb3) in various cells. The detection of Gb3 deposits may help in the diagnosis. To date, no immunofluorescence‐specific detection of Gb3 in conjunctival biopsies has been reported.
Paula Adriana Rozenfeld +1 more
exaly +3 more sources
AbstractBackground: Fabry disease is an X‐linked disorder due to a deficiency of α‐galactosidase A and leads to the accumulation of globotriaosylceramide (Gb3) in various cells. The detection of Gb3 deposits may help in the diagnosis. To date, no immunofluorescence‐specific detection of Gb3 in conjunctival biopsies has been reported.
Paula Adriana Rozenfeld +1 more
exaly +3 more sources
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
openaire +2 more sources
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
openaire +2 more sources
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
openaire +1 more source
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
openaire +1 more source
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
openaire +2 more sources
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
openaire +2 more sources
Monitoring enzyme replacement therapy in Fabry disease—Role of urine globotriaosylceramide
Journal of Inherited Metabolic Disease, 2005SummaryAnderson‐Fabry disease (referred to as Fabry disease) is an X‐linked disorder characterized by a deficiency of the lysosomal enzyme α‐galactosidase A and the subsequent accumulation in various tissues of globotriaosylceramide (Gb3), the main substrate of the defective enzyme.
Whitfield, Phillip D. +10 more
openaire +3 more sources