Literature DB >> 4269276

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

J N Thompson, A C Stoolmiller, R Matalon, A Dorfman.   

Abstract

Extracts of cultured normal human skin fibroblasts released radioactivity from a (14)C-labeled heptasaccharide prepared by addition of [(14)C]N-acetylgalactosamine to the nonreducing terminus of a hexasaccharide derived from chondroitin 4-sulfate whereas fibroblast extracts from patients with Tay-Sachs and Sandhoff-Jatzkewitz diseases did not. The results suggest that N-acetyl-beta-hexosaminidase A is responsible for degradation of the oligosaccharide substrate.

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Year:  1973        PMID: 4269276     DOI: 10.1126/science.181.4102.866

Source DB:  PubMed          Journal:  Science        ISSN: 0036-8075            Impact factor:   47.728


  10 in total

Review 1.  Biochemistry and genetics of gangliosidoses.

Authors:  K Sandhoff; H Christomanou
Journal:  Hum Genet       Date:  1979       Impact factor: 4.132

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

Authors:  J L Stirling
Journal:  Biochem J       Date:  1974-08       Impact factor: 3.857

Review 3.  Adventures in viscous solutions.

Authors:  A Dorfman
Journal:  Mol Cell Biochem       Date:  1974-08-01       Impact factor: 3.396

4.  Diagnosis of Tay-Sachs disease using radiolabelled chondroitin 6-sulphate-derived trisaccharides.

Authors:  T Yutaka; T Kato; S Okada; M Midorikawa; H Yabuuchi
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

5.  Activity of procollagen-prolyl-hydroxylase and N-acetyl-beta-glucosaminidase in liver biopsies from patients with chronic liver diseases.

Authors:  G Pott; G Eberhardt; U Gerlach
Journal:  Klin Wochenschr       Date:  1979-06-01

6.  Characterization of unusual hexosaminidase A (HEX A) deficient human mutants.

Authors:  J S O'Brien; L Tennant; M L Veath; C R Scott; W E Bucknall
Journal:  Am J Hum Genet       Date:  1978-11       Impact factor: 11.025

7.  Clearance of human N-acetyl-beta-hexosaminidases from rat circulation.

Authors:  T Bearpark; J L Stirling
Journal:  Biochem J       Date:  1977-12-15       Impact factor: 3.857

8.  A difference in the specificities of human liver N-acetyl-beta-hexosaminidases A and B detected by their activities towards glycosaminoglycan oligosaccharides.

Authors:  T M Bearpark; J L Stirling
Journal:  Biochem J       Date:  1978-09-01       Impact factor: 3.857

9.  Degradation of keratan sulphate by beta-N-acetylhexosaminidases A and B.

Authors:  T Ludolph; E Paschke; J Glössl; H Kresse
Journal:  Biochem J       Date:  1981-03-01       Impact factor: 3.857

10.  Association of NAG levels with specific psychiatric symptoms in unipolar patients.

Authors:  M J Garvey; M Noel
Journal:  J Psychiatry Neurosci       Date:  1995-01       Impact factor: 6.186

  10 in total

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