Literature DB >> 95964

Further studies on hepatic acidic glycosaminoglycans in the Hurler syndrome.

J A Kofoed, A A Tocci, A C Barceló.   

Abstract

Acid GAG were isolated from hepatic tissue from 3 patients with Hurler syndrome and 3 normal controls. Gross elevations in the uronic acid and hexosamine contents were found in Hurler livers compared with the normal ones. The total GAG concentration was significantly increased (about 25fold) in Hurler patients.

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Year:  1978        PMID： 95964     DOI： 10.1007/bf01936968

Source DB:  PubMed          Journal:  Experientia        ISSN： 0014-4754


  12 in total

1.  DISTINCTION AMONG FOUR FORMS OF HURLER'S SYNDROME.

Authors:  K TERRY; A LINKER
Journal:  Proc Soc Exp Biol Med       Date:  1964-02

2.  A modified uronic acid carbazole reaction.

Authors:  T BITTER; H M MUIR
Journal:  Anal Biochem       Date:  1962-10       Impact factor: 3.365

3.  The determination of amino sugars in the presence of amino acids and glucose.

Authors:  C CESSI; F PILIEGO
Journal:  Biochem J       Date:  1960-12       Impact factor: 3.857

4.  The estimation of carbohydrates in plant extracts by anthrone.

Authors:  E W YEMM; A J WILLIS
Journal:  Biochem J       Date:  1954-07       Impact factor: 3.857

5.  Hurler's syndrome.

Authors:  K MEYER; P HOFFMAN
Journal:  Arthritis Rheum       Date:  1961-10

6.  Structural studies on heparitin sulfate of normal and Hurler tissues.

Authors:  J Knecht; J A Cifonelli; A Dorfman
Journal:  J Biol Chem       Date:  1967-10-25       Impact factor: 5.157

7.  Gargoylism (mucopolysaccharidosis type II). Accumulation of glycosaminoglycans, gangliosides and glycoproteins and activity of some related glycolytic enzymes in liver, spleen and brain.

Authors:  P A Roukema; C H Oderkerk; G van den Berg
Journal:  Clin Chim Acta       Date:  1973-03-30       Impact factor: 3.786

8.  Hurler's syndrome, an -L-iduronidase deficiency.

Authors:  R Matalon; A Dorfman
Journal:  Biochem Biophys Res Commun       Date:  1972-05-26       Impact factor: 3.575

9.  Hepatic acid mucopolysaccharides in the mucopolysaccharidoses type I, II and 3.

Authors:  B A Gordon; M D Haust
Journal:  Clin Biochem       Date:  1971-10       Impact factor: 3.281

10.  Hurler's, Hunter's and Morquio's syndromes. A biochemical study in the light of current views of the underlying defects.

Authors:  M F Dean; H Muir; R J Ewins
Journal:  Biochem J       Date:  1971-08       Impact factor: 3.857
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