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Literature DB >> 7496144

Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria.

F Irie¹, H Murakoshi, T Suzuki, Y Suzuki, K Kon, S Ando, K Yoshida, Y Hirabayashi.

Abstract

We previously reported for the first time two Japanese patients with aspartylglycosaminuria (AGU). A novel disialo Asn N-glycoside (AG-5) has been isolated from the urine of one of the patients in addition to four known monosialo Asn N-glycosides (AG-1 to AG-4) by gel filtration and anion exchange chromatography in this study. Final purification of AG-5 was achieved by an electrochemical chromatographic method, high performance liquid chromatography with pulsed amperometric detector (HPLC-PAD). The yield of AG-5 was approximately 1 mg l-1 urine. The chemical structures of AG-1 to AG-5 were characterized by gas-liquid chromatography, a permethylation study, fast atom bombardment-mass spectrometry (FAB-MS), and nuclear magnetic resonance (NMR). Based on the structural analysis, AG-5 had the following novel structure: NeuAc alpha 2-->8NeuAc alpha 2-->3Gal beta 1-->4GlcNAc beta 1-->Asn.

Entities: Chemical Disease Gene Mutation Species

Mesh：

Substances：

Year: 1995 PMID： 7496144 DOI： 10.1007/bf00731332

Source DB: PubMed Journal: Glycoconj J ISSN： 0282-0080 Impact factor: 2.916

27 in total

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Authors: S HAKOMORI
Journal: J Biochem Date: 1964-02 Impact factor: 3.387

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Journal: Eur J Biochem Date: 1976-08-01

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Journal: FEBS Lett Date: 1976-10-15 Impact factor: 4.124

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Authors: R J Pollitt; F A Jenner; H Merskey
Journal: Lancet Date: 1968-08-03 Impact factor: 79.321

5. A ganglioside of rat ascites hepatoma AH 7974F cells. Occurrence of a novel disialoganglioside (GD1 alpha) with a unique N-acetylneuraminosyl (alpha 2-6)-N-acetylgalactosamine structure.

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Journal: J Biol Chem Date: 1986-03-05 Impact factor: 5.157

6. Splicing defect of the glycoasparaginase gene in two Japanese siblings with apartylglucosaminuria.

Authors: K Yoshida; N Yanagisawa; A Oshima; H Sakuraba; Y Iida; Y Suzuki
Journal: Hum Genet Date: 1992 Sep-Oct Impact factor: 4.132

7. Primary structure determination of five sialylated oligosaccharides derived from bronchial mucus glycoproteins of patients suffering from cystic fibrosis. The occurrence of the NeuAc alpha(2----3)Gal beta(1----4)[Fuc alpha(1----3)] GlcNAc beta(1----.) structural element revealed by 500-MHz 1H NMR spectroscopy.

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Journal: J Biol Chem Date: 1984-07-25 Impact factor: 5.157

8. Separation of fucosylated oligosaccharides using high-pH anion-exchange chromatography with pulsed-amperometric detection.

Authors: M R Hardy; R R Townsend
Journal: Carbohydr Res Date: 1989-06-01 Impact factor: 2.104

Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria.

1. A RAPID PERMETHYLATION OF GLYCOLIPID, AND POLYSACCHARIDE CATALYZED BY METHYLSULFINYL CARBANION IN DIMETHYL SULFOXIDE.

2. Structural determination of three glycoasparagines isolated from the urine of a patient with aspartylglycosaminuria.

3. Characterization of a mannose-containing glycoasparagine isolated from urine of a patient with aspartylglycosylaminuria (AGU).

4. Aspartylglycosaminuria. An inborn error of metabolism associated with mental defect.

5. A ganglioside of rat ascites hepatoma AH 7974F cells. Occurrence of a novel disialoganglioside (GD1 alpha) with a unique N-acetylneuraminosyl (alpha 2-6)-N-acetylgalactosamine structure.

6. Splicing defect of the glycoasparaginase gene in two Japanese siblings with apartylglucosaminuria.

8. Separation of fucosylated oligosaccharides using high-pH anion-exchange chromatography with pulsed-amperometric detection.

9. Clinical and biochemical delineation of aspartyl-glycosaminuria as observed in two members of an Italian family.

10. Isolation and characterization of the sulfated gangliotriaosylceramide from rat kidney.

1. Sleep disturbances in aspartylglucosaminuria (AGU): a questionnaire study.

Review 2. Exploration of the Sialic Acid World.