Primary Structure of Three Mannosyl-glycoasparagines and Nine Sialyl-oligosaccharides Isolated from the Urine of Two Patients with Gaucher's Disease (Infantile Form)

Montreuil, Jean; Strecker, Gérard; Halbeek, Herman van; Dorland, L.; Vliegenthart, Johannes F.G.; Cartigny, Bernard; Farriaux, J. P.

doi:10.1111/j.1432-1033.1983.tb07373.x

Cited by 11 publications

(4 citation statements)

References 23 publications

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“…With respect to the sphingolipidoses, oligosaccharide excretion as a direct consequence of the enzyme deÐciency is seen in GM1gangliosidosis and Sandho † disease (Gravel et al 1995 ;Suzuki et al 1995). Increased excretion of oligosaccharide in Gaucher disease has been described in two patients with the infantile neuronopathic form probably as a consequence of obstruction of lysosomal functioning due to the massive glycolipid accumulation in the lysosomes (Michalski et al 1983). In this paper we describe increased excretion of oligosaccharide in adult non-neuronopathic patients.…”

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confidence: 75%

“…There were no similarities seen between the patterns obtained from Gaucher urines and that from a sialidosis urine (Figure 1b). Storage of glycoprotein material in spleen and liver (DeGasperi et al 1990) is apparently a secondary phenomenon due to obstruction of lysosomal enzymes, caused by the massive storage of glucosylceramide (Michalski et al 1983). DeGasperi and colleagues (1990) detected intense staining of Gaucher cells in liver and spleen with lectins that react with N-linked mannose-and fucose-containing oligosac-charide, with N-acetyllactosamine sequences therein and with terminal galactose and neuraminic acid residues.…”

Section: Discussionmentioning

confidence: 99%

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Oligosaccharide excretion in adult Gaucher disease

Jong¹,

Aerts²,

Weely³

et al. 1998

J of Inher Metab Disea

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Gaucher disease is a lysosomal storage disease characterized by storage of glucocerebroside due to lysosomal glucocerebrosidase deficiency. Increased urinary excretion of sialyloligosaccharides and mannosylglycoasparagines has been described for two patients with the infantile form of the disease, probably as a consequence of obstruction of lysosomal functioning due to the glycolipid accumulation in lysosomes. By thin-layer chromatography, we found increased urinary oligosaccharide excretion in a series of adult non-neuronopathic patients. Oligosaccharide patterns were comparable between patients and also with the pattern observed in infantile Gaucher disease. Composition was analysed by methanolysis and gas chromatography. Mannose and N-acetylglucosamine are the main carbohydrates in all oligosaccharide bands. A statistically significant correlation was found between oligosaccharide excretion and the severity of the disease expressed as severity score index. Patients treated with enzyme replacement therapy showed a reduction up to 65% of the original oligosaccharide excretion after 1 year of treatment, comparable with the reduction in spleen volume.

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confidence: 75%

Section: Discussionmentioning

confidence: 99%

Oligosaccharide excretion in adult Gaucher disease

Jong¹,

Aerts²,

Weely³

et al. 1998

J of Inher Metab Disea

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“…Man ' GlcNAc # Asn (M6) was prepared by exhaustive Pronase digestion of ovalbumin followed by cation-exchange chromatography on Dowex 50i2, 200-400 mesh, H + form (1.5 cmi150 cm) by the method of Tai et al [16]. Man & GlcNAc # Asn was isolated from the urine of a patient with Gaucher's disease as previously described [17]. Man & GlcNAc # (M5) was obtained by hydrazinolysis followed by re-N-acetylation [18].…”

Section: Oligosaccharides and Glycoasparaginesmentioning

confidence: 99%

Interleukin 2 is a lectin that associates its receptor with the T-cell receptor complex

Zanetta

Alonso

Michalski

1996

Biochemical Journal

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To determine the nature of the mechanism by which the binding of interleukin-2 (IL-2) to its receptor (IL-2R beta) induces IL-2R beta phosphorylation by the tyrosine kinase p56lck associated with the T-cell receptor (TCR) complex, we investigated the possibility that this mechanism was due to the putative lectin activity of IL-2 ([Sherblom, Sathyamoorthy, Decker and Muchmore (1989) J. Immunol. 143, 939-944]. Here we demonstrate that IL-2 is a calcium-independent lectin specific for oligomannosidic N-glycans with five and six mannose residues. This lectin activity is preserved after binding of IL-2 to IL-2R beta. IL-2 behaves as a bifunctional molecule that associates IL-2R beta with specific glycoprotein ligands of the TCR complex including a glycosylated form of CD3.

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“…For example, in urine of Gaucher’s disease the presence has been demonstrated of Man 5 , lacking the Man residues D 1 , D 2 , D 3 and C ; Man 4 lacking the Man residues D 1 , D 2 , D 3 , C and 4 , and Man 2 lacking the Man residues D 1 , D 2 , D 3 , C , 4 , A and B . 30) In all cases the reporter group signals allow the complete assignment of structures. The partial structures arise from the catabolic route and are helpful in reconstructing the pathway of the stepwise degradation.…”

Section: Introductionmentioning

confidence: 99%

The complexity of glycoprotein-derived glycans

Vliegenthart

2017

Proceedings of the Japan Academy. Ser. B: Physical and Biologic

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A brief review is presented of our studies on the structure of glycoprotein-derived glycans. The emphasis is on the introduction of high-resolution 1H-NMR spectroscopy for the unambiguous determination of primary structures. For this purpose, we developed the structural reporter group concept. Structural reporters are defined as unique markers of structural elements in the NMR spectra. Application of this concept led to the discovery of numerous new structures. Furthermore, a number of structures presented in the literature could be corrected. The results are relevant for insight in the various steps in glycan metabolism in health and disease, for the function and mode of action of glycans in vivo and for the interpretation of structural information obtained through other techniques. The strength of the approach is further shown for several highly complex glycoproteins, carrying very heterogeneous and complicated glycans.

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Primary Structure of Three Mannosyl-glycoasparagines and Nine Sialyl-oligosaccharides Isolated from the Urine of Two Patients with Gaucher's Disease (Infantile Form)

Cited by 11 publications

References 23 publications

Oligosaccharide excretion in adult Gaucher disease

Oligosaccharide excretion in adult Gaucher disease

Interleukin 2 is a lectin that associates its receptor with the T-cell receptor complex

The complexity of glycoprotein-derived glycans

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