Oligosaccharides accumulated in the bovine β -mannosidosis kidney
Identifieur interne : 001A62 ( Main/Exploration ); précédent : 001A61; suivant : 001A63Oligosaccharides accumulated in the bovine β -mannosidosis kidney
Auteurs : M. Z. Jones [États-Unis] ; E. J. S. Rathke [États-Unis] ; D. A. Gage [États-Unis] ; C. E. Costello [États-Unis] ; K. Murakami [Japon] ; M. Ohta [Japon] ; F. Matsuura [Japon]Source :
- Journal of Inherited Metabolic Disease [ 0141-8955 ] ; 1992-01-01.
Abstract
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.
Url:
DOI: 10.1007/BF01800344
Affiliations:
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<front><div type="abstract" xml:lang="en">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.</div>
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