fucosidosis/carbohydrate

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Patterns of complex carbohydrate storage in four lysosomal storage diseases: fucosidosis, GM1-gangliosidosis, GM2-gangliosidosis (Sandhoff variant) and mannosidosis.

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Structure of the three major fucosyl-glycoasparagines accumulating in the urine of a patient with fucosidosis.

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Fifteen fucosyl-oligosaccharides and fucosyl-glycoasparagines have been isolated from the urine of a patient with fucosidosis. The structure of the three most abundant glycoasparagines are as follows: alpha-Fuc-(1 lead to 6)-beta-GlcNAc-Asn; alpha-Man-(1 leads to 6)-beta-Man-(1 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

Structure of the accumulating oligosaccharide in fucosidosis.

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Two water-soluble complex carbohydrate storage products were isolated from tissues and urine of patients with an inherited deficiency of lysosomal alpha-L-fucosidase (fucosidosis). The major component was an oligosaccharide of approximate molecular weight 1700, indicating that it was a

Glycoprotein lysosomal storage disorders: alpha- and beta-mannosidosis, fucosidosis and alpha-N-acetylgalactosaminidase deficiency.

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Glycoproteinoses belong to the lysosomal storage disorders group. The common feature of these diseases is the deficiency of a lysosomal protein that is part of glycan catabolism. Most of the lysosomal enzymes involved in the hydrolysis of glycoprotein carbohydrate chains are exo-glycosidases, which

Pulsed amperometric detection of carbohydrates in lysosomal storage disease fibroblasts: a new screening technique for carbohydrate storage diseases.

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A first step in determining the metabolic defect in patients with an unknown storage disease is to identify the stored material. In the case of fibroblasts storing carbohydrates, this can be accomplished by trifluoroacetic acid (TFA) hydrolysis producing monosaccharides which are separated by

Characterization of SSEA-1 glycolipids from the brain of a patient with fucosidosis.

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Neutral glycolipids from the brain of a patient with Fucosidosis were analyzed and two complex glycolipids containing five and eight sugars were isolated from the cortical grey matter. These two glycolipids reacted with antibodies recognizing the SSEA-1 [Le(x)(X)] carbohydrate determinant. SSEA-1

Urinary abnormalities in fucosidosis. Characterization of a disaccharide and two glycoasparagines.

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The urinary excretion of fucose-containing material was found to be highly increased in a patient with fucosidosis type 2. Three structurally related compounds, a disaccharide and two glycoasparagines, were isolated from the urine. The isolation procedure included ultrafiltration, gel chromatography

Biosynthesis, processing, and extracellular release of alpha-L-fucosidase in lymphoid cell lines of different genetic origins.

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In humans, the quantity of alpha-L-fucosidase in serum is determined by heredity. The mechanism controlling levels of the enzyme in serum is unknown. Lymphoid cell lines derived from individuals with either low, intermediate, or high alpha-L-fucosidase in serum were established. Steady-state levels

Fractionation of L-fucose-containing oligosaccharides on immobilized Aleuria aurantia lectin.

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The carbohydrate-binding specificity of Aleuria aurantia lectin was investigated by analyzing the behavior of a variety of fucose-containing oligosaccharides on an A. aurantia lectin-Sepharose column. Studies with complex-type oligosaccharides obtained from various glycoproteins by hydrazinolysis

The core-specific lysosomal alpha(1-6)-mannosidase activity depends on aspartamidohydrolase activity.

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The substrate specificity of the core-specific rat liver lysosomal alpha(1-6)-mannosidase was investigated using mannosylated oligosaccharides and glycoasparagines. Hydrolysis of Man(alpha 1-6) linkage hydrolysis was demonstrated to follow the action of endoglycosidases, namely

Human liver alpha-L-fucosidase. Purification, characterization, and immunochemical studies.

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Human liver alpha-L-fucosidase has been purified 6300-fold to apparent homogeneity with 66% yield by a two-step affinity chromatographic procedure utilizing agarose epsilon-aminocaproyl-fucosamine. Isoelectric focusing revealed that all six isoelectric forms of the enzyme were purified.

A capillary electrophoresis procedure for the screening of oligosaccharidoses and related diseases.

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The most widely used method for the biochemical screening of oligosaccharidoses is the analysis of the urinary oligosaccharide pattern by thin-layer chromatography on silica gel plates. However, this method is not always sensitive enough, and it is extremely time-consuming and laborious. In this

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