Literature DB >> 4455225

NN'-diacetylchitobiase activity in Tay-Sachs disease and Sandhoff's disease.

J L Stirling.   

Abstract

In Tay-Sachs disease and Sandhoff's disease respectively, one of the N-acetyl-beta-hexosaminidases (form A) or both (forms A and B) are absent, but glycosaminoglycans containing N-acetylhexosamines are not accumulated. The presence of NN'-diacetylchitobiase in livers from patients with these diseases raises the possibility that this new enzyme is involved in the metabolism of glycosaminoglycans rather than the enzymes previously described.

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Year:  1974        PMID： 4455225      PMCID： PMC1168118          DOI： 10.1042/bj1410597

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  12 in total

1.  OLIGOSACCHARASE ACTIVITY OF BETA-N-ACETYL-D-GLUCOSAMINIDASE OF BEEF LIVER.

Authors:  B WEISSMANN; S HADJIIOANNOU; J TORNHEIM
Journal:  J Biol Chem       Date:  1964-01       Impact factor: 5.157

2.  Human N-acetyl-beta-hexosaminidases: hydrolysis of N, N' diacetylchitobiose by a low molecular weight enzyme.

Authors:  J L Stirling
Journal:  FEBS Lett       Date:  1974-02-15       Impact factor: 4.124

3.  Mucopoly saccharide storage in organs of a patient with Sandhoff's disease.

Authors:  D A Applegarth; G Bozoian
Journal:  Clin Chim Acta       Date:  1972-06       Impact factor: 3.786

4.  Separation of chitin oligosaccharides by thin-layer chromatography.

Authors:  R F Powning; H Irzykiewicz
Journal:  J Chromatogr       Date:  1967-07

5.  Ganglioside storage diseases.

Authors:  J S O'Brien; S Okada; M W Ho; D L Fillerup; M L Veath; K Adams
Journal:  Fed Proc       Date:  1971 May-Jun

6.  [Oligosaccharide excretion in a case of GM 2 gangliosidosis due to total N-acetylhexosaminidase deficiency].

Authors:  G Strecker; J Montreuil
Journal:  Clin Chim Acta       Date:  1971-07       Impact factor: 3.786

7.  Beta-galactosidase, beta-glucosidase and N-acetyl-beta-glucosaminidase in human kidney.

Authors:  N Dance; R G Price; D Robinson; J L Stirling
Journal:  Clin Chim Acta       Date:  1969-05       Impact factor: 3.786

8.  Sanfilippo syndrome: profound deficiency of alpha-acetylglucosaminidase activity in organs and skin fibroblasts from type-B patients.

Authors:  J S O'Brien
Journal:  Proc Natl Acad Sci U S A       Date:  1972-07       Impact factor: 11.205

9.  N-acetyl-beta-hexosaminidase: role in the degradation of glycosaminoglycans.

Authors:  J N Thompson; A C Stoolmiller; R Matalon; A Dorfman
Journal:  Science       Date:  1973-08-31       Impact factor: 47.728

10.  N-Acetyl-beta-glucosaminidases in human spleen.

Authors:  D Robinson; J L Stirling
Journal:  Biochem J       Date:  1968-04       Impact factor: 3.857
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  1 in total

Review 1.  Glycosphingolipid hydrolases: properties and molecular genetics.

Authors:  M Wan Ho; A G Norden; J A Alhadeff; J S O'Brien
Journal:  Mol Cell Biochem       Date:  1977-10-07       Impact factor: 3.396
  1 in total

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