caa a22 4500 469043768 CHVBK 20180323132818.0 cr unu---uuuuu 170328e19920101xx s 000 0 eng 10.1007/BF01800344 doi (NATIONALLICENCE)springer-10.1007/BF01800344 Oligosaccharides accumulated in the bovine β -mannosidosis kidney [Elektronische Daten] [M. Jones, E. Rathke, D. Gage, C. Costello, K. Murakami, M. Ohta, F. Matsuura] Summary: The phenotype of bovineβ-mannosidosis (β-mannosidase deficiency), recently identified in Salers cattle, is similar to the caprine form of the disease (Abbittet al., 1991). This investigation was designed to characterize accumulated kidney oligosaccharides in bovineβ-mannosidosis. Oligosaccharides were extracted from the kidney of an affected Salers calf and purified by chromatographic techniques. The amount of accumulating oligosaccharides in 1 g of wet tissue was about 21µmol. Structures of derivatized oligosaccharides were characterized by high-performance liquid chromatography, mass spectrometry, methylation analysis and sequential exoglycosidase digestions. The major accumulating oligosaccharides were Manβ1-4GlcNAc and Manβ1-4GlcNAcβ1-4GlcNAc. Oligosaccharides accumulating in minor amounts were Manβ1-4GlcNAcβ1-4Manβ1-4GlcNAc, Manα1-6Manβ1-4GlcNAcβ1-4GlcNAc and Manβ1-4GlcNAcβ1-4Manβ1-4GlcNAcβ1-4GlcNAc. As in caprineβ-mannosidosis, oligosaccharides with terminalβ-mannose residues and cleaved as well as uncleaved chitobiose linkages were identified in bovineβ-mannosidosis kidney. The accumulating oligosaccharides in tissue were thus identical in bovine and caprineβ-mannosidosis; however, the source of the novel oligosaccharides remains to be determined. SSIEM and Kluwer Academic Publishers, 1992 Jones M. Department of Pathology, Michigan State University, East Lansing, MI, USA aut Rathke E. Department of Pathology, Michigan State University, East Lansing, MI, USA aut Gage D. Department of Biochemistry, Michigan State University, East Lansing, MI, USA aut Costello C. Department of Chemistry, Massachusetts Institute of Technology, Cambridge, MA, USA aut Murakami K. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut Ohta M. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut Matsuura F. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 57-67 0141-8955 15:1<57 1992 15 10545 https://doi.org/10.1007/BF01800344 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800344 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Jones M. Department of Pathology, Michigan State University, East Lansing, MI, USA aut NATIONALLICENCE 700 1- Rathke E. Department of Pathology, Michigan State University, East Lansing, MI, USA aut NATIONALLICENCE 700 1- Gage D. Department of Biochemistry, Michigan State University, East Lansing, MI, USA aut NATIONALLICENCE 700 1- Costello C. Department of Chemistry, Massachusetts Institute of Technology, Cambridge, MA, USA aut NATIONALLICENCE 700 1- Murakami K. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut NATIONALLICENCE 700 1- Ohta M. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut NATIONALLICENCE 700 1- Matsuura F. Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima, Japan aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 57-67 0141-8955 15:1<57 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer