Oligosaccharides accumulated in the kidney of a goat with β-mannosidosis: Mass spectrometry of intact permethylated derivatives

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Abstract

Two oligosaccharides accumulate in the kidney of a goat with β-mannosidosis. These oligosaccharides were isolated and purified from kidney extracts by Bio-Gel P2 gel permeation column chromatography. Their structures were characterized as Manβ1 → 4GlcNAc and Manβ1 → 4G1cNAcβ1 → 4G1cNAc by mass spectrometry of the permethylated intact oligosaccharide alcohols and permethylated native oligosaccharides. Carbohydrate composition analysis, methylation linkage studies, and enzymatic hydrolysis were also performed. Stored in 1 g of kidney were 1.6 μmol of disaccharide and 7.6 μmol of trisaccharide, which was three times that found in the brain of this affected animal (M. Z. Jones and R. A. Laine, 1981, J. Biol. Chem., 256, 5181–5184). In both the brain and kidney of the affected goat, oligosaccharide accumulation was evidently represented by membrane-bound, electron-lucent vacuoles in numerous cell types. While lesions in the brain were associated with profound neurological deficits, functional impairment of the kidney was not apparent. Similar oligosaccharides excreted in urine may be derived from those stored in the kidney. The mass spectrometric methods utilized in this investigation will facilitate comparison of oligosaccharide composition in different tissues and biological samples in β-mannosidosis and other disorders of glycoprotein catabolism.

References (39)

  • M.Z. Jones et al.

    J. Biol. Chem

    (1981)
  • M.Z. Jones et al.

    J. Biol. Chem

    (1981)
  • B. Kjellman et al.

    J. Pediat

    (1969)
  • P.-A. Ockerman

    Lancet

    (1967)
  • N.E. Norden et al.

    J. Biol. Chem

    (1973)
  • N.E. Norden et al.

    FEBS Lett

    (1973)
  • G. Strecker et al.

    Biochimie

    (1976)
  • K. Yamashita et al.

    J. Biol. Chem

    (1980)
  • F. Matsuura et al.

    Arch. Biochem. Biophys

    (1981)
  • A. Kobata et al.

    J. Biol. Chem

    (1972)
  • K. Stellner et al.

    Arch. Biochem. Biophys

    (1973)
  • H. Bjorndal et al.

    Carbohydr. Res

    (1967)
  • G.O.H. Schwarzmann et al.

    Carbohydr. Res

    (1974)
  • J. Karkkainen

    Carbohydr. Res

    (1970)
  • J. Karkkainen

    Carbohydr. Res

    (1971)
  • J. Karkkainen

    Carbohydr. Res

    (1971)
  • M.Z. Jones et al.

    Soc. Neurosci. Abst

    (1979)
  • J.H. Whittem et al.

    J. Pathol. Bacteriol

    (1957)
  • R.D. Jolly

    J. Pathol

    (1971)
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    This work was supported in part by Grant NS16886 to M. Z. J., Grants GM23902 and AM25101 to R. A. L., and Grants AM12434 and RR00480 to Charles C. Sweeley.

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