mucopolysaccharidoses/carbohydrate

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Disease-specific non-reducing end carbohydrate biomarkers for mucopolysaccharidoses.

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A considerable need exists for improved biomarkers for differential diagnosis, prognosis and monitoring of therapeutic interventions for mucopolysaccharidoses (MPS), inherited metabolic disorders that involve lysosomal storage of glycosaminoglycans. Here we report a simple, reliable method based on

[Detection of mucopolysaccharidosis and mucolipidosis by quantitative and qualitative analyses of urinary complex carbohydrates].

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Diagnostic screening for mucopolysaccharidoses types I-VII by fluorophore-labelled carbohydrate PAGE.

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N-butyldeoxynojirimycin treatment restores the innate fear response and improves learning in mucopolysaccharidosis IIIA mice.

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Mucopolysaccharidosis IIIA is a heritable neurodegenerative disorder resulting from the dysfunction of the lysosomal hydrolase sulphamidase. This leads to the primary accumulation of the complex carbohydrate heparan sulphate in a wide range of tissues and the secondary neuronal storage of

Studies on the composition of urinary glycosaminoglycans and oligosaccharides in patients with mucopolysaccharidoses who were receiving fibroblast transplants.

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This communication reports studies on the composition of the urinary glycosaminoglycans and oligosaccharides in mucopolysaccharidosis patients who were being treated by fibroblast transplantation. The urinary glycosaminoglycans were precipitated with 9-aminoacridine, the oligosaccharides remaining

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

Structural determination of oligosaccharides from recombinant iduronidase released with peptide N-glycanase F using fluorophore-assisted carbohydrate electrophoresis.

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The lysosomal storage disorder mucopolysaccharidoses I (MPS I) is caused by a deficiency in the production of alpha-L-iduronidase. Recently, a recombinant alpha-L-iduronidase has been produced in Chinese hamster ovary (CHO) cells. It is thought that for alpha-L-iduronidase to be correctly targeted

Vasculopathic and cardiomyopathic changes induced by low-protein high-carbohydrate tapioca based diet in bonnet monkey. Vasculopathic and cardiomyopathic changes in induced malnutrition.

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Mucoid vasculopathy is an entity characterized by a generalized mucopolysaccharidosis of blood vessels. It has been observed in patients from our Institute. Bonnet monkeys (Macaca radiata) were given low-protein normal-carbohydrate and low-protein high-carbohydrate tapioca based diets for

Catabolic disorders of complex carbohydrates.

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The various disorders caused by heritable defects in complex carbohydrate catabolism comprise two groups: (A) The mucopolysaccharidoses, six main and several subtypes are described. (B) The mucolipidoses (oligosaccharidoses), at least nine types being recognized. Whilst most of these are now well

Modulation of carbohydrate metabolism during carcinogenesis.

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Investigations in a number of experimental models and some observations in humans have indicated that changes in carbohydrate metabolism precede the development of many tumor types and appear to be closely associated, if not causally related, to neoplastic cell transformation. Thus, a decrease or

Biochemical evidence for superior correction of neuronal storage by chemically modified enzyme in murine mucopolysaccharidosis VII.

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Enzyme replacement therapy has been used successfully in many lysosomal storage diseases. However, correction of brain storage has been limited by the inability of infused enzyme to cross the blood-brain barrier (BBB). We recently reported that PerT-GUS, a form of β-glucuronidase (GUS) chemically

Skeletal modifications in mucopolysaccharidoses: an overview.

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The mucopolysaccharidoses (MPS) are a group of rare diseases characterized by deficiencies in different enzymes required for degradation of complex carbohydrates. The enzymatic deficiencies lead to lysosomal accumulation of dermatan sulphate, heparan sulphate, and keratan sulphate in different

Heparan sulfate proteoglycans: The sweet side of development turns sour in mucopolysaccharidoses.

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Heparan sulfate proteoglycans (HSPGs) are complex carbohydrate-modified proteins ubiquitously expressed on cell surfaces, extracellular matrix and basement membrane of mammalian tissues. Beside to serve as structural constituents, they regulate multiple cellular activities. A critical involvement of

Generation of an induced pluripotent stem cell line (TRNDi005-A) from a Mucopolysaccharidosis Type IVA (MPS IVA) patient carrying compound heterozygous p.R61W and p.WT405del mutations in the GALNS gene.

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Mucopolysaccharidosis type IVA (MPS IVA) is a rare genetic disease caused by mutations in the GALNS gene and is inherited in an autosomal recessive manner. GALNS encodes N-acetylgalactosamine-6-sulfatase that breaks down certain complex carbohydrates known as glycosaminoglycans (GAGs). Deficiency in

Two-dimensional NMR spectroscopy of urinary glycosaminoglycans from patients with different mucopolysaccharidoses.

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Patients with different types of mucopolysaccharidoses (MPS) lack specific lysosomal enzymes, which leads to tissue accumulation and urinary excretion of glycosaminoglycans (GAGs). Since little is known about the molecular composition of the excreted GAG fragments, we used two-dimensional

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