Literature DB >> 2116550

Quantitative analysis of disaccharides in the urine of beta-mannosidosis patients.

S Tjoa1, D A Wenger, P V Fennessey.   

Abstract

Until recently, there have not been any confirmed reports of beta-mannosidase deficiency in man. We have now analysed urine from two patients with confirmed beta-mannosidase deficiency and have found Man-beta(1-4)GlcNAc concentrations of 65 and 73 mg/mmol creatinine. These levels are at least 12 times higher than those found in patients with other lysosomal storage diseases. The method we report for this analysis requires only 0.1 ml of urine and uses a gas chromatograph, area ratio, calibration curve for the quantitative analysis.

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Year:  1990        PMID： 2116550     DOI： 10.1007/bf01799685

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  6 in total

1.  Quantitative metabolic profiling of urinary organic acids by gas chromatography-mass spectrometry: comparison of isolation methods.

Authors:  J A Thompson; S P Markey
Journal:  Anal Chem       Date:  1975-07       Impact factor: 6.986

2.  Fast atom bombardment/collisional activation mass spectrometry of beta-D-mannosyl-(1----4)-beta-D-N-acetylglucosaminyl (1----N) urea and related compounds.

Authors:  W Heerma; J W Dallinga; L Dorland; A M van Rhee; J van Pelt; M Duran
Journal:  Rapid Commun Mass Spectrom       Date:  1988-05       Impact factor: 2.419

3.  Human beta-mannosidase deficiency.

Authors:  D A Wenger; E Sujansky; P V Fennessey; J N Thompson
Journal:  N Engl J Med       Date:  1986-11-06       Impact factor: 91.245

4.  Determination of aminosugar linkages in glycolipids by methylation. Aminosugar linkages of ceramide pentasaccharides of rabbit erythrocytes and of Forssman antigen.

Authors:  K Stellner; H Saito; S I Hakomori
Journal:  Arch Biochem Biophys       Date:  1973-04       Impact factor: 4.013

5.  Isolation and characterisation of 4-O-beta-D-mannopyranosyl-2-acetamido- 2-deoxy-D-glucose from the urine of a patient with mucolipidosis II and its occurrence in normal and pathological urine.

Authors:  M Lemonnier; C Derappe; L Poenaru; M A Chester; A Lundblad; S Svensson; P A Ockerman
Journal:  FEBS Lett       Date:  1982-05-17       Impact factor: 4.124

6.  Structure of a new hexasaccharide from the coaggregation polysaccharide of Streptococcus sanguis 34.

Authors:  F C McIntire; C A Bush; S S Wu; S C Li; Y T Li; M McNeil; S S Tjoa; P V Fennessey
Journal:  Carbohydr Res       Date:  1987-08-15       Impact factor: 2.104
  6 in total
  1 in total

1.  Bovine kidney beta-mannosidase: purification and characterization.

Authors:  B L Sopher; C E Traviss; K T Cavanagh; M Z Jones; K H Friderici
Journal:  Biochem J       Date:  1993-01-15       Impact factor: 3.857
  1 in total

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