Oligosaccharides accumulated in the bovineβ‐mannosidosis kidney

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Abstract
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.