Literature DB >> 942387

Isolation of the liver N-aspartyl-beta-glucosaminidase in aspartylglucosaminuria.

H Savolainen.   

Abstract

The isolation of liver N-aspartyl-beta-glucosaminidase in human aspartylglucosaminuria, where this enzyme activity is diminished, yields an enzyme molecule with the same molecular weight and pH optimum as the normal enzyme. Its activity is 10% of that of the control preparation. Combination of both enzymes results in the summation of both activities, and the pathological enzyme does not inhibit the control preparation. It is concluded that no change into a totally different isoenzyme has occurred in aspartylglucosaminuria.

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Year:  1976        PMID: 942387      PMCID: PMC1172648          DOI: 10.1042/bj1530749

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


  8 in total

1.  Studies on N-aspartyl-beta-glucosaminidase in aspartylglycosaminuria.

Authors:  H Somer; J Palo; H Savolainen; A Konttinen
Journal:  Clin Chim Acta       Date:  1975-04-16       Impact factor: 3.786

2.  Estimation of molecular weights of small proteins on polyacrylamide gel electrophoresis.

Authors:  A Gonenne; J Lebowitz
Journal:  Anal Biochem       Date:  1975-04       Impact factor: 3.365

3.  DISC ELECTROPHORESIS. II. METHOD AND APPLICATION TO HUMAN SERUM PROTEINS.

Authors:  B J DAVIS
Journal:  Ann N Y Acad Sci       Date:  1964-12-28       Impact factor: 5.691

4.  A modified colorimetric method for the estimation of N-acetylamino sugars.

Authors:  J L REISSIG; J L STORMINGER; L F LELOIR
Journal:  J Biol Chem       Date:  1955-12       Impact factor: 5.157

5.  Protein measurement with the Folin phenol reagent.

Authors:  O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal:  J Biol Chem       Date:  1951-11       Impact factor: 5.157

6.  Aspartylglycosaminuria. An inborn error of metabolism associated with mental defect.

Authors:  R J Pollitt; F A Jenner; H Merskey
Journal:  Lancet       Date:  1968-08-03       Impact factor: 79.321

7.  Glycoasparagine metabolites in patients with aspartylglycosaminuria: comparison between English and Finnish patients with special reference to storage materials.

Authors:  J Palo; R J Pollitt; K M Pretty; H Savolainen
Journal:  Clin Chim Acta       Date:  1973-08-17       Impact factor: 3.786

8.  Distribution, purification and properties of 1-aspartamido-beta-N-acetylglucosamine amidohydrolase.

Authors:  J Conchie; I Strachan
Journal:  Biochem J       Date:  1969-12       Impact factor: 3.857

  8 in total
  4 in total

1.  Purification and some properties of 1-aspartamido-beta-N-acetylglucosamine amidohydrolase from human liver.

Authors:  B Dugal; J Stromme
Journal:  Biochem J       Date:  1977-09-01       Impact factor: 3.857

2.  Isolation of a human hepatic 60 kDa aspartylglucosaminidase consisting of three non-identical polypeptides.

Authors:  M Baumann; L Peltonen; P Aula; N Kalkkinen
Journal:  Biochem J       Date:  1989-08-15       Impact factor: 3.857

Review 3.  Aspartylglycosaminuria: an inborn error of glycoprotein catabolism.

Authors:  C P Maury
Journal:  J Inherit Metab Dis       Date:  1982       Impact factor: 4.982

4.  Purification and structure of human liver aspartylglucosaminidase.

Authors:  J W Rip; M B Coulter-Mackie; C A Rupar; B A Gordon
Journal:  Biochem J       Date:  1992-12-15       Impact factor: 3.857

  4 in total

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