aspartylglucosaminuria/carbohydrate

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Aspartylglycosaminuria: a generalized storage disease. Morphological and histochemical studies.

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Ingia / Ingia

Aspartylglycosaminuria (AGU) is a hereditary metabolic disorder characterized by slowly progressive mental deterioration from infancy, urinary excretion of large amounts of aspartylglycosamine, and decreased activity of the lysosomal enzyme aspartylglcosamine amido hydrolase in various body tissues

Aspartylglycosaminuria: protein chemistry and molecular biology of the most common lysosomal storage disorder of glycoprotein degradation.

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Ingia / Ingia

Aspartylglycosaminuria (AGU) (McKusick 20840) is the most common disorder of glycoprotein degradation caused by the failure of lysosomes to digest the protein-to-carbohydrate linkage of Asn-linked glycoproteins. During the past few years there has been significant progress in our understanding of

Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease.

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Ingia / Ingia

Eighty-five clinical urine samples and nineteen urine samples previously found by other laboratories to suggest genetic metabolic defects were prepared for trimethylsilylation by treatment with urease, followed by azeotropic dehydration. The "Target Analyte Search" program provided with the VG Trio

Glycosaparaginase from human leukocytes. Inactivation and covalent modification with diazo-oxonorvaline.

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Ingia / Ingia

The apparent active site of human leukocyte glycoasparaginase (N4-(beta-acetylglucosaminyl)-L-asparaginase EC 3.5.1.26) has been studied by labeling with an asparagine analogue, 5-diazo-4-oxo-L-norvaline. Glycoasparaginase was purified 4,600-fold from human leukocytes with an overall recovery of

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

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Ingia / Ingia

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

Identification of 4-N-2-acetamido-2-deoxy-beta-D-glucopyranosyl-L-asparagine in biological materials by gas chromatography-mass spectrometry.

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Ingia / Ingia

A specific and sensitive method for the identification of 4-N-2-acetamido-2-deoxy-beta-D-glucopyranosyl-L-asparagine (GlcNAc-Asn) in urine in aspartylglycosaminuria and in hydrolysates of glycoproteins is described. The method involves permethylation of GlcNAc-Asn followed by gas

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

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Ingia / Ingia

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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Herbal & Natural Medicine

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