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.
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Jones, M.Z., Rathke, E.J.S., Gage, D.A. et al. Oligosaccharides accumulated in the bovineβ-mannosidosis kidney. J Inherit Metab Dis 15, 57–67 (1992). https://doi.org/10.1007/BF01800344
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DOI: https://doi.org/10.1007/BF01800344