mucolipidoses/carbohydrate

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I-cell disease. A hypothesis for the structure of the carbohydrate recognition site on beta-D-N-acetylhexosaminidase.

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I-cell disease (mucolipidosis II) is presented as a model for endo- and exo-cytosis phenomena in man. A hypothesis is presented for the structure of the carbohydrate recognition site on fibroblast-derived beta-D-N-acetylhexosaminidase that may extend to the other affected hydrolases and that is

Carbohydrate composition of purified serum glycoproteins in mucolipidosis II and mucolipidosis III.

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Mucolipidosis II (I-cell disease) and Mucolipidosis III (ML III) are inherited disorders in which the molecular defect may involve an abnormality in a common post-translational modification step (possibly glycosylation) shared by lysosomal hydrolases. We tested whether such an alteration might be a

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

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Mucolipidosis III beta-N-acetyl-D-hexosaminidase A. Purification and properties.

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Mucolipidosis III acid hydrolases possess an altered carbohydrate recognition marker needed for their lysosomal localization. As a result of this alteration, a portion of these enzymes is secreted from the cell to the extracellular spaces. The structural changes that may have occurred to one of

A study of highly purified mucolipidosis III urinary N-acetyl-beta-D-hexosaminidase B.

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Highly purified N-acetyl-beta-D-hexosaminidase B from normal urine and urine of a patient with mucolipidosis III was used to determine whether it has undergone any of the alterations associated with this genetic defect. Examination by sodium dodecyl sulphate/polyacrylamide gel electrophoresis showed

Light and heavy lysosomes: characterization of N-acetyl-beta-D-hexosaminidase isolated from normal and I-cell disease lymphoblasts.

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We previously reported that I-cell disease lymphoblasts maintain normal or near-normal intracellular levels of lysosomal enzymes, even though N-acetylglucosamine-1-phosphotransferase activity is severely depressed or absent (Little et al., Biochem. J., 248, 151-159, 1987). The present study,

Upregulation of Sortilin, a Lysosomal Sorting Receptor, Corresponds with Reduced Bioavailability of Latent TGFβ in Mucolipidosis II Cells.

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Mucolipidosis II (ML-II) is a lysosomal disease caused by defects in the carbohydrate-dependent sorting of soluble hydrolases to lysosomes. Altered growth factor signaling has been identified as a contributor to the phenotypes associated with ML-II and other lysosomal disorders but an understanding

Abnormal urinary excretion of sialoglycoconjugates in patients with mucolipidosis.

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Low molecular sialoglycoconjugates were isolated by the charcoal adsorption method from the urines of patients with mucolipidosis (one patient with mucolipidosis type I variant and three patients with mucolipidosis type II). The sialoglycoconjugates were fractionated into two major fractions (SG-1

Mannose 6-phosphate-independent targeting of lysosomal enzymes in I-cell disease B lymphoblasts.

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B lymphocytes from patients with I-cell disease (ICD) maintain normal cellular levels of lysosomal enzymes despite a deficiency of the enzyme UDP-N-acetylglucosamine: lysosomal enzyme N-acetylglucosamine-1-phosphotransferase. We find that an ICD B lymphoblastoid cell line targets about 45% of the

Isolation of acidic glycopeptides from urine by means of anion-exchange resins. Application to some cases of glycosphingolipidosis or mucolipidosis.

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An acidic fraction containing aminosugar was isolated by means of Dowex 1 from normal human urine which had previously been filtered through Ecteolacellulose. After purification, the fraction was shown to be composed of peptides and carbohydrates in comparable amounts. Threonine, serine and

Biosynthesis, processing, and secretion of alpha-L-fucosidase in lymphoid cells from patients with I-cell disease and pseudo-Hurler polydystrophy.

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N-Acetylglucosamine 1-phosphotransferase is a key enzyme required for synthesis of the mannose 6-phosphate recognition marker that is used by many newly made acid hydrolases for their transport to lysosomes. It has previously been found that lymphoid cells from patients with I-cell disease and

I-Cell disease: isoelectric focusing, concanavalin A-Sepharose 4B binding and kinetic properties of human liver acid beta-D-galactosidases.

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Isoelectric focusing of the acid beta-D-galactosidases (beta-D-galactoside galactohydrolase, EC 3.2.1.23) in normal crude liver supernatant fluids demonstrated multiple isoelectric forms in the pH range 4.58-5.15, while corresponding I-cell disease samples showed an absence of isoelectric forms in

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

Excretion-reuptake route of beta-hexosaminidase in normal and I-cell disease cultured fibroblasts.

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It has been proposed that in cultured fibroblasts the final packaging of enzymes in lysosomes requires excretion followed by pinocytosis by neighboring cells via a carbohydrate-specific receptor mechanism. It has also been proposed that the abnormally high activity of lysosomal enzymes in the medium

Mucolipidosis I--a sialidosis.

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Mucolipidosis I is characterized by Hurler-like features and skeletal dysplasia with a cherry-red macular spot and signs of neurodegeneration involving neuronal cells and myelin. Excessive amounts of sialic acid-containing compounds were found in cultured fibroblasts, leukocytes, and urine of a

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