Literature DB >> 6422155

The simple detection of neuraminic acid-containing urinary oligosaccharides in patients with glycoprotein storage diseases.

A C Sewell.   

Abstract

Urine samples from patients with different types of glycoprotein storage disease were chromatographed by gel filtration and the fractions analysed for sialic acid. Patients with mucolipidoses I and II excreted the largest amounts of bound sialic acid. One patient with GM1 gangliosidosis showed an abnormal level of sialyloligosaccharide excretion. Other patients showed normal results. With the present method mucolipidoses I and II, together with GM1 gangliosidosis, are readily distinguished from other possible oligosaccharidurias.

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Year:  1983        PMID： 6422155     DOI： 10.1007/bf02310870

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


  25 in total

1.  The determination of sugar in blood and spinal fluid with anthrone reagent.

Authors:  J H ROE
Journal:  J Biol Chem       Date:  1955-01       Impact factor: 5.157

2.  Macular cherry-red spots and myoclonus with dementia: coexistent neuraminidase and beta-galactosidase deficiencies.

Authors:  D A Wenger; T J Tarby; C Wharton
Journal:  Biochem Biophys Res Commun       Date:  1978-05-30       Impact factor: 3.575

3.  Structure of nine sialyl-oligosaccharides accumulated in urine of eleven patients with three different types of sialidosis. Mucolipidosis II and two new types of mucolipidosis.

Authors:  G Strecker; M C Peers; J C Michalski; T Hondi-Assah; B Fournet; G Spik; J Montreuil; J P Farriaux; P Maroteaux; P Durand
Journal:  Eur J Biochem       Date:  1977-05-16

4.  Mucolipidosis type IV: ganglioside sialidase deficiency.

Authors:  G Bach; M Zeigler; T Schaap; G Kohn
Journal:  Biochem Biophys Res Commun       Date:  1979-10-29       Impact factor: 3.575

5.  Urinary sialic acid levels in aspartylglycosaminuria.

Authors:  C P Maury
Journal:  Clin Chim Acta       Date:  1981-01-22       Impact factor: 3.786

6.  Mucolipidosis IV, a sialolipidosis due to ganglioside sialidase deficiency.

Authors:  L Caimi; G Tettamanti; B Berra; F Omodeo Sale; C Borrone; R Gatti; P Durand; J J Martin
Journal:  J Inherit Metab Dis       Date:  1982       Impact factor: 4.982

7.  The cherry red spot-myoclonus syndrome: a newly recognized inherited lysosomal storage disease due to acid neuraminidase deficiency.

Authors:  J S O'Brien
Journal:  Clin Genet       Date:  1978-07       Impact factor: 4.438

8.  Urinary oligosaccharide screening in patients with beta-galactosidase deficiency.

Authors:  A C Sewell; J Gehler; J Spranger
Journal:  Eur J Pediatr       Date:  1980-05       Impact factor: 3.183

9.  Clinical and biochemical delineation of aspartyl-glycosaminuria as observed in two members of an Italian family.

Authors:  J Gehler; A C Sewell; C Becker; J Hartmann; J Spranger
Journal:  Helv Paediatr Acta       Date:  1981

10.  Mucolipidosis I--a sialidosis.

Authors:  J Sphranger; J Gehler; M Cantz
Journal:  Am J Med Genet       Date:  1977
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  2 in total

1.  Normomorphic sialidosis in two female adults with severe neurologic disease and without sialyl oligosacchariduria.

Authors:  K Harzer; M Cantz; A C Sewell; S S Dhareshwar; W Roggendorf; R W Heckl; O Schofer; R Thumler; J Peiffer; W Schlote
Journal:  Hum Genet       Date:  1986-11       Impact factor: 4.132

2.  Salla disease in one non-Finnish patient.

Authors:  B Echenne; M Vidal; I Maire; J C Michalski; P Baldet; J Astruc
Journal:  Eur J Pediatr       Date:  1986-09       Impact factor: 3.183
  2 in total

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