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J. Biochem, 1976, Vol. 80, No. 2 195-201
© 1976 Japanese Biochemical Society


research-article

Characterization of One Neutral and Two Acidic Glycoasparagines Isolated from the Urine of Patients with Aspartylglycosylaminuria (AGU)1

Kazuyuki SUGAHARA, Susumu FUNAKOSHI, Ikuo FUNAKOSHI, Pertti AULA* and Ikuo YAMASHINA2

Department of Biological Chemistry, Faculty of Pharmaceutical Sciences, Kyoto University Sakyo-ku, Kyoto, Kyoto 606
*Department of Pathology, University of Helsinki Helsinki, Finland

2 Correspondence should be addressed to I. Yamashina.

One neutral and two acidic glycoasparagines were isolated from the urine of patients with aspartylglycosylaminuria (AGU). The neutral one was identified as ß-Gal-(1{uparrow}4)-ß-GICNAc-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 [EC] ] from Clostridium perfringens. The structure of the resulting sialic acid-free glycoasparagine was determined to be ß-Gal-ß-GIcNAc-ß-Gal-(1{uparrow}4)-B-GlcNAc-Asn based mainly on the results of sequential enzymatic degradations.

1 This study was supported by a Grant-in-Aid for Scientific Research from the Ministry of Education, Science and Culture, of Japan.


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