Abstract |
Two water-soluble glycoasparagine storage products were isolated from the liver of a patient with inherited deficiency of lysosomal N-aspartyl-beta- glucosaminidase ( aspartylglycosaminuria). The compounds were characterized using gas chromatographic-mass spectrometric analyses of intact and specifically degraded derivatives. The storage compounds were identified as 2-acetamido-1-N-(4'-L-aspartyl)-2-deoxy-beta-D-glucopyranosylamine and alpha-D-mannopyranosyl-(1 leads to 6)-beta-D-mannopyranosyl-(1 leads to 4)-2-acetamido-2-deoxy-beta-D-glucopyranosyl-(1 leads to 4)-2-acetamido-1-N-(4'-L-aspartyl)-2-deoxy-beta-D-glucopyranosylamine. Their amounts in the pathological liver were approximately 2.7 mg/g and 0.2 mg/g, wet weight, respectively. These glycoasparagines are structurally related to the inner core region of a number of different glycoproteins.
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Authors | P Maury |
Journal | The Journal of biological chemistry
(J Biol Chem)
Vol. 254
Issue 5
Pg. 1513-5
(Mar 10 1979)
ISSN: 0021-9258 [Print] United States |
PMID | 762149
(Publication Type: Journal Article)
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Chemical References |
- Glycopeptides
- Oligosaccharides
- glycoasparagines
- Asparagine
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Topics |
- Asparagine
(analogs & derivatives, metabolism)
- Chromatography, Gas
- Glycopeptides
(metabolism)
- Humans
- Liver
(metabolism)
- Mass Spectrometry
- Mucolipidoses
(metabolism)
- Oligosaccharides
(metabolism)
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