1977
DOI: 10.1111/j.1432-1033.1977.tb11540.x
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Structure of Nine Sialyl-Oligosaccharides Accumulated in Urine of Eleven Patients with Three Different Types of Sialidosis. Mucolipidosis II and Two New Types of Mucolipidosis

Abstract: Urines of 11 patients with three different types of mucolipidosis characterized by a total or partial sialidase deficiency, were studied. In all cases, we found an important accumulation of sialyl‐oligosaccharides. The structure of 9 of them has been determined: α‐AcNeu‐(2→3)‐β‐Gal‐(1→4)‐β‐GlcNAc‐(1→2)‐α‐Man‐(1→3)‐β‐Man‐(1→4)‐GlcNAc, α‐AcNeu‐(2→6)‐β‐Gal‐(1→4)‐β‐GlcNAc‐(1→2)‐α‐Man‐(1→3)‐β‐Man‐(1→4)‐GlcNAc, α‐AcNeu‐(2→6)‐β‐Gal‐(1→4)‐β‐GlcNAc‐(1→2)‐α‐Man‐(1→3)[α‐Man‐(1→6)]β‐Man‐(1→4)‐GlcNAc, α‐AcNeu‐(2→3)‐β‐Gal‐(… Show more

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Cited by 137 publications
(44 citation statements)
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“…The deficiency of enzymes responsible for this lysosomal turnover of glycoproteins causes incomplete degradation of N-glycosidically linked oligosaccharides, and results in accumulations of these oligosaccharides in the urine and the tissues. In sialidosis, increased excretion of urinary oligosaccharides was reported in previous papers (Strecker et al 1977;Dorland et al 1978;Strecker and Michalski 1978;Kuriyama et al 1981). Our investigations in a severe infantile sialidosis patient with abdominal ascites have revealed virtually identical constitutional profiles of oligosaccharides in both the urine and in the abdominal ascites, and have revealed much higher levels of excretions of oligosaccharides in the urine than in the abdominal ascites.…”
Section: Discussionsupporting
confidence: 80%
“…The deficiency of enzymes responsible for this lysosomal turnover of glycoproteins causes incomplete degradation of N-glycosidically linked oligosaccharides, and results in accumulations of these oligosaccharides in the urine and the tissues. In sialidosis, increased excretion of urinary oligosaccharides was reported in previous papers (Strecker et al 1977;Dorland et al 1978;Strecker and Michalski 1978;Kuriyama et al 1981). Our investigations in a severe infantile sialidosis patient with abdominal ascites have revealed virtually identical constitutional profiles of oligosaccharides in both the urine and in the abdominal ascites, and have revealed much higher levels of excretions of oligosaccharides in the urine than in the abdominal ascites.…”
Section: Discussionsupporting
confidence: 80%
“…1) was identical to that obtained for sialidosis urine [16]. The levels of these oligosaccharides are significantly increased as compared to normal urine (see Table 1); however, they are considerably lower than those in the urine of sialidosis patients [16].…”
Section: Characterization Of Sialyl-oligosaccharidrssupporting
confidence: 76%
“…In particular, the change in chemical shift of H-2 of mannose-3 from 6 = 4.076 ppin to 4.251 ppm, in conjunction with the presence of an additional set of H-1 and H-2 signals for a terminal a-(1 + 3)-linked mannose residue (for sialidosis, GM,-gangliosidosis or mannosidosis [16,24,251, The quantities of glycoasparagines are very low and cannot be detected by direct TLC of urine. Nevertheless, their excretion is significant, because these glycopeptides have never previously becn described in either normal urine or urine from other types of lysosomal diseases.…”
Section: Discussionmentioning
confidence: 99%
“…‡ To whom correspondence should be addressed: viously described (19,(25)(26)(27)(28). Glycopeptides were obtained from different glycoproteins by extensive Pronase digestion followed by Biogel P2 chromatography (29).…”
Section: Biochemicals-recombinant Human Cytokines (Produced In Bacteria)mentioning
confidence: 99%