Literature DB >> 187576

Characterization of one neutral and two acidic glycoasparagines isolated from the urine of patients with aspartylglycosylaminuria (AGU).

K Sugahara, S Funakoshi, I Funakoshi, P Alla, I Yamashina.   

Abstract

One neutral and two acidic glycoasparagines were isolated from the urine of patients with aspartylglycosylaminuria (AGU). The neutral one was identified as beta-Gal-(1 leads to 4)-beta-GlcNAc-Asn. The acidic ones were composed of 1 mole of sialic acid and 2 moles each of galactose and N-acetylglucosamine, attached to asparagine, and were isomeric with respect to the position of sialic acid attachment since they produced the same glycoasparagine on incubation with the neuraminidase [EC 3.2.1.18] from Clostridium perfringens. The structure of the resulting sialic acid-free glycoasparagine was determined to be beta-Gal-beta-GlcNAc-beta-Gal-(1 leads to 4)-beta-GlcNAc-Asn based mainly on the results of sequential enzymatic degradations.

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Year:  1976        PMID： 187576     DOI： 10.1093/oxfordjournals.jbchem.a131264

Source DB:  PubMed          Journal:  J Biochem        ISSN： 0021-924X            Impact factor:   3.387


  5 in total

Review 1.  Aspartylglycosaminuria: an inborn error of glycoprotein catabolism.

Authors:  C P Maury
Journal:  J Inherit Metab Dis       Date:  1982       Impact factor: 4.982

2.  Amniotic fluid glycoasparagines in fetal aspartylglycosaminuria.

Authors:  I Mononen; V Kaartinen; T Mononen
Journal:  J Inherit Metab Dis       Date:  1988       Impact factor: 4.982

3.  Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency: new mutations and the paradox between genotype and phenotype.

Authors:  J L Keulemans; A J Reuser; M A Kroos; R Willemsen; M M Hermans; A M van den Ouweland; J G de Jong; R A Wevers; W O Renier; D Schindler; M J Coll; A Chabas; H Sakuraba; Y Suzuki; O P van Diggelen
Journal:  J Med Genet       Date:  1996-06       Impact factor: 6.318

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

Authors:  F Irie; H Murakoshi; T Suzuki; Y Suzuki; K Kon; S Ando; K Yoshida; Y Hirabayashi
Journal:  Glycoconj J       Date:  1995-06       Impact factor: 2.916

Review 5.  Exploration of the Sialic Acid World.

Authors:  Roland Schauer; Johannis P Kamerling
Journal:  Adv Carbohydr Chem Biochem       Date:  2018-11-28       Impact factor: 12.200
  5 in total

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