gangliosidoses/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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Brain glycoproteins in GM1-gangliosidosis: isolation and carbohydrate composition of glycopeptides.

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Characteristics of asparagine-linked sugar chains of sphingolipid activator protein 1 purified from normal human liver and GM1 gangliosidosis (type 1) liver.

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Asparagine-linked sugar chains of sphingolipid activator protein 1 (SAP-1) purified from normal human liver and GM1 gangliosidosis (type 1) liver were comparatively investigated. Oligosaccharides released from the two SAP-1 samples by hydrazinolysis were fractionated by paper electrophoresis and by

Lectin histochemistry of gangliosidosis. II. Neurovisceral tissues from patients with Sandhoff's disease.

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Lectin histochemical studies were performed on selected formalin-fixed, paraffin-embedded tissues of patients affected with the O variant of GM2-gangliosidosis (i.e., Sandhoff's disease). The purpose was to identify specific sugar residues of undegraded "stored" substances in cytoplasm of affected

Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis).

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The carbohydrate composition was determined for ceramide hexosides isolated from brains of patients with Tay-Sachs disease and generalized gangliosidosis (hereby named GM1-gangliosidosis). Gray matter of patients with each disease showed a characteristic abnormal ceramide hexoside pattern. In

Prenatal lesions in an ovine fetus with GM1 gangliosidosis.

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Sheep affected with ovine GM1 gangliosidosis are normal at birth and develop clinical signs, initially ataxia, commencing at approximately 5 months of age, which progresses rapidly to recumbency. Superovulation and embryo transfer techniques were applied to a flock of carrier sheep of ovine GM1

Application of lectin histochemistry and carbohydrate analysis to the characterization of lysosomal storage diseases.

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In lysosomal storage diseases that involve a defect in the catabolism of glycoconjugates, lectin histochemistry adds a new dimension to the characterization of stored carbohydrates as it identifies sugar residues in situ in the affected cells and, thus, determines which cell types are affected by

Lectin histochemistry of gangliosidosis. I. Neural tissue in four mammalian species.

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Lectin histochemical studies were performed on paraffin-embedded brain, spinal cord and eye sections of 16 animals from four different species affected with GM1- and GM2-gangliosidosis to identify specific carbohydrate residues in the perikaryon of neurons. We examined tissues from cats, cattle and

The role of lysosomal sialidase and beta-galactosidase in processing the complex carbohydrate chains on lysosomal enzymes and possibly other glycoproteins.

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Previous studies using the lectin RCA-I from Ricinus communis have indicated that several lysosomal enzymes in the fibroblasts of patients deficient in beta-galactosidase carry excess terminal galactose. Electrophoretic studies have shown that the same enzymes and the non-lysosomal adenosine

Morphological analysis of corneal opacity in Shiba dog with GM1 gangliosidosis.

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GM1 gangliosidosis is one of the inherited metabolic lysosomal storage disorders characterized by neurological symptoms caused by beta-galactosidase deficiency and consequent accumulation of GM1 ganglioside in neuronal cells. Shiba dogs affected with GM1 gangliosidosis have been found to suffer from

Infantile gangliosidoses: Mapping a timeline of clinical changes.

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Infantile gangliosidoses include GM1 gangliosidosis and GM2 gangliosidosis (Tay-Sachs disease, Sandhoff disease). To date, natural history studies in infantile GM2 (iGM2) have been retrospective and conducted through surveys. Compared to iGM2, there is even less natural history information available

Towards enzyme replacement in GM2 gangliosidosis: organ disposition and induced central nervous system uptake of human beta-hexosaminidase in the cat.

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The rapid plasma clearance of human placental beta-hexosaminidase in the cat is due mainly to a receptor-mediated mechanism recognizing terminal N-acetyl glucosaminyl and mannosyl residues on glycoproteins. Using a sensitive single radial immunodiffusion assay, specific for human

Beta-hexosaminidase isozymes and replacement therapy in Gm2 gangliosidosis.

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The problem of cell targeting of lysosomal enzymes is a critical one in the development of strategies for therapeutic enzyme replacement in lysosomal storage diseases. In principle, posttranscriptional isozymes with different carbohydrate-chain composition may be helpful in exploiting existing

Multiple carbohydrate-cleaving specificities in human acidic and neutral glycosidases.

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The common identity of human acidic beta-D-glucosidase (beta-D-glucoside glucohydrolase, EC 3.2.1.21) and beta-D-xylosidase (1,4-beta-D-xylan xylohydrolase, EC 3.2.1.37) as one enzyme and that of acidic beta-D-galactosidase (beta-D-galactoside galactohydrolase, EC 3.2.1.23), beta-D-fucosidase (no

Lending a helping hand, screening chemical libraries for compounds that enhance beta-hexosaminidase A activity in GM2 gangliosidosis cells.

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Enzyme enhancement therapy is an emerging therapeutic approach that has the potential to treat many genetic diseases. Candidate diseases are those associated with a mutant protein that has difficulty folding and/or assembling into active oligomers in the endoplasmic reticulum. Many lysosomal storage

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