Characterization of dermatan sulfate and heparan sulfate in the urine of a patient with the hunter syndrome.

Kimura, Atsushi; Hayashi, Shiro; Tsurumi, Koichi

doi:10.1620/tjem.131.227

Cited by 5 publications

(7 citation statements)

References 11 publications

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“…This disagreement will be accounted for by the finding that CPC could not completely precipitate GAG with Mr less than 3000 under our experimental conditions (Kimura et al 1980a). …”

Section: Excretion Patterns Of Gagmentioning

confidence: 76%

“…was unrelated and not subjected to the enzymatic examination. Some urinary HS fractions obtained from patients with MPS I-H/S and II (Kimura et al 1980a(Kimura et al , 1982 were also used.…”

Section: Methodsmentioning

confidence: 99%

“…The methods for the following were described previously (Kimura et al 1980a) ; the electrophoresic determination of urinary HS : DS : CS ratio, the isolation and fractionation of GAG by Dowex 1 and Sephadex G-50 chromatography, the identification of the fractionated GAG by electrophoresis and enzymatic digestion, the determination of Mr by gel filtration, and the chemical analysis unless stated otherwise.…”

Section: Methodsmentioning

confidence: 99%

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Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.

Kimura

Hayashi

Koseki

et al. 1984

Tohoku J. Exp. Med.

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Urinary heparan sulfates (HS) from two siblings with mucopolysaccharidosis (MPS) III-B were fractionated by chromatography with Dowex 1 and Sephadex G-50. Their Mr ranged from 1600 to 8000, and 95% of them were included in the region less than 5000. Fractions with lower Mr contained larger amounts of 0-and N-sulfates. The chemical analysis and deaminative cleavage of HS suggested that an intact HS molecule was composed of some blocks rich in G1cNAc and G1cUA and other blocks rich in G1cNS, IdUA and 0-sulfate. G1cNAc-UA-G1cNS-UAG1cNAc-UA-G1cNAc was found to be a major oligosaccharide of HS with Mr less than 1800. Trisaccharides, G1cNAc-G1cUA-aMan and G1cNAc-IdUA-aMan, were released from the nonreducing end of HS-oligosaccharides by deaminative cleavage. They carried 0-3 moles of ester sulfate. G1cNAc-IdUA-aMan was more sulfated than the other. The release of significant amounts of nonsulfated trisaccharide conform to the enzyme defect in this disease. Urinary HS obtained from another patient with MPS III were examined by the same way. Although the patient was not examined enzymatically, the structure of urinary GAG suggested a defect of a-N-acetylglucosaminidase in the patient.Sanfilippo syndrome ; mucopolysaccharidosis III-B ; urinary glycosaminoglycan ; heparan sulfate ; oligosaccharide structure Patients with MPS excrete in their urine an excessive amount of GAG with low Mr, which is a favorable material to study the structure or catabolic process of GAG. In our previous studies, it was shown that the urinary DS from patients with Hurler-Scheie syndrome (MPS I-H/S) had an IdUA residue at the nonreducing end, and only 50% of excess sulfate were bound to inner IdUA residues

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“…This disagreement will be accounted for by the finding that CPC could not completely precipitate GAG with Mr less than 3000 under our experimental conditions (Kimura et al 1980a). …”

Section: Excretion Patterns Of Gagmentioning

confidence: 76%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.

Kimura

Hayashi

Koseki

et al. 1984

Tohoku J. Exp. Med.

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“…Furthermore , the chemical analysis indicated that in the DS of the largest molecular weight (2.0 M-1 fr,) only 10 per cent of sulfate were excess, whereas in the DS of the smallest molecular weight (2.0 M-3 fr.) about 50 per cent of sulfate were excess (Kimura et al 1980). That may be ascribed to an uneven distribution of iduronosulfate residues in their original molecules; the former may be derived from the part poor and the latter from the part rich in the residue .…”

Section: Disaccharides Derived From the Hunter Urine Dsmentioning

confidence: 99%

“…In the preceding paper (Kimura et al 1980), we demonstrated that a patient with the Hunter syndrome excreted a large amount of DS-and HS-fragments which were low in molecular weight and rich in sulfate content, and speculated that the excess sulfate groups, presumably bound to iduronate residues, obstracted the degradation processes of DS and HS through their inhibitory action for iduronidase.…”

mentioning

confidence: 96%

Chemical structure of urinary dermatan sulfate excreted by a patient with the hunter syndrome.

Kimura

Hayashi

Tsurumi

1980

Tohoku J. Exp. Med.

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Investigation of GAGS on 24-hour and 2-hour urines from calcium oxalate stone formers and healthy subjects

Gianotti

Genestar

Palou

et al. 1989

International Urology and Nephrology

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The role of urinary glycosaminoglycans (GAGS) in calcium oxalate lithiasis is of great interest in urologic research. It has been claimed that GAGS are important inhibitors of calcium oxalate crystal growth and aggregation. The aim of this paper is to evaluate GAGS excretion and concentration in two groups of patients, calcium stone formers (with or without metabolic alteration) and a control group, to detect possible differences. The findings of this study show that significant differences exist not only in the 24-hour average excretion between stone formers without alteration and healthy subjects but also in the mean concentration values between the stone former and control groups. The same results are obtained from the 2-hour urine analysis. It is concluded that 2-h urine analyses of GAGS have the same or more practical value than a 24-h urine analysis and that the results must be expressed in terms of concentration.

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Characterization of dermatan sulfate and heparan sulfate in the urine of a patient with the hunter syndrome.

Cited by 5 publications

References 11 publications

Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.

Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.

Chemical structure of urinary dermatan sulfate excreted by a patient with the hunter syndrome.

Investigation of GAGS on 24-hour and 2-hour urines from calcium oxalate stone formers and healthy subjects

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