fabry disease/carbohydrate

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Ability of Lactobacillus fermentum to overcome host alpha-galactosidase deficiency, as evidenced by reduction of hydrogen excretion in rats consuming soya alpha-galacto-oligosaccharides.

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BACKGROUND Soya and its derivatives represent nutritionally high quality food products whose major drawback is their high content of alpha-galacto-oligosaccharides. These are not digested in the small intestine due to the natural absence of tissular alpha-galactosidase in mammals. The passage of

Receptor-mediated endocytosis of α-galactosidase A in human podocytes in Fabry disease.

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Injury to the glomerular podocyte is a key mechanism in human glomerular disease and podocyte repair is an important therapeutic target. In Fabry disease, podocyte injury is caused by the intracellular accumulation of globotriaosylceramide. This study identifies in the human podocyte three endocytic

The molecular defect leading to Fabry disease: structure of human alpha-galactosidase.

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Fabry disease is an X-linked lysosomal storage disease afflicting 1 in 40,000 males with chronic pain, vascular degeneration, cardiac impairment, and other symptoms. Deficiency in the lysosomal enzyme alpha-galactosidase (alpha-GAL) causes an accumulation of its substrate, which ultimately leads to

MRI and MRS findings in fucosidosis; a rare lysosomal storage disease.

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Fucosidosis is a rare lysosomal storage disorder caused by deficient activity of the enzyme l-fucosidase in all tissues. We presented magnetic resonance imaging [MRI] and MR spectroscopy [MRS] findings of a 4-year-old boy with genetically proven fucosidosis. He had a history and clinical findings of

Ionization and fragmentation of neutral and acidic glycosphingolipids with a Q-TOF mass spectrometer fitted with a MALDI ion source.

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This paper reports the use of a quadrupole time-of-flight (Q-TOF) mass spectrometer fitted with a matrix-assisted laser desorption/ionization (MALDI) ion source for the analysis of neutral and acidic glycosphingolipids. All compounds gave strong [M + Na]+ ions with 2,5-dihydroxybenzoic acid as the

Glycosidases: inborn errors of glycosphingolipid catabolism.

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Glycosphingolipids (GSLs) are information-rich glycoconjugates that occur in nature mainly as constituents of biomembranes. Each GSL contains a complex carbohydrate chain linked to a ceramide moiety that anchors the molecule to biomembranes. In higher animals, catabolism of GSLs takes place in

Expression and characterization of glycosylated and catalytically active recombinant human alpha-galactosidase A produced in Pichia pastoris.

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Fabry disease is an X-linked inborn error of glycolipid metabolism caused by deficiency of the lysosomal enzyme alpha-galactosidase A. This enzyme is responsible for the hydrolysis of terminal alpha-galactoside linkages in various glycolipids. An improved method of production of recombinant

The 1.9 A structure of alpha-N-acetylgalactosaminidase: molecular basis of glycosidase deficiency diseases.

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In the lysosome, glycosidases degrade glycolipids, glycoproteins, and oligosaccharides. Mutations in glycosidases cause disorders characterized by the deposition of undegraded carbohydrates. Schindler and Fabry diseases are caused by the incomplete degradation of carbohydrates with terminal

A historical perspective of the glycosphingolipids and sphingolipidoses.

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Glycosphingolipids are a polysaccharide chain between 1 and 40 carbohydrate residues long glycosidically linked to ceramide (a long-chain aliphatic amino-alcohol or sphingoid) that is embedded in the cell plasma membrane with the carbohydrate moiety on the outside. The sphingoid imparts rigidity to

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