Literature DB >> 4236799

Generalized gangliosidosis: impaired cleavage of galactose from a mucopolysaccharide and a glycoprotien.

M C MacBrinn, S Okada, M W Ho, C C Hu, J S O'Brien.   

Abstract

We have demonstrated a tenfold (or greater) reduction of the cleavage of galactose from a mucopolysaccharide and a glycoprotein in generalized gangliosidosis, a lysosomal beta-galactosidase deficiency disease. The bony abnormalities in this disease may be due to defective catabolism of mucopolysaccharides in connective tissue.

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Year:  1969        PMID： 4236799     DOI： 10.1126/science.163.3870.946

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


  10 in total

Review 1.  Progress in investigations of sphingolipidoses.

Authors:  M Adachi; L Schneck; B W Volk
Journal:  Acta Neuropathol       Date:  1978-08-07       Impact factor: 17.088

Review 2.  Mucopolysaccharidoses and mucolipidoses.

Authors:  F Van Hoof
Journal:  J Clin Pathol Suppl (R Coll Pathol)       Date:  1974

3.  Purified human liver acid beta-D-galactosidases possessing activity towards G(M1)-ganglioside and lactosylceramide.

Authors:  A L Miller; R G Frost; J S O'Brien
Journal:  Biochem J       Date:  1977-09-01       Impact factor: 3.857

4.  Fibroblast culture and inherited neurological diseases.

Authors:  G Stewart
Journal:  Proc R Soc Med       Date:  1971-02

5.  The genetic mucolipidoses. Diagnosis and differential diagnosis.

Authors:  J W Spranger; H R Wiedemann
Journal:  Humangenetik       Date:  1970

Review 6.  Progress in birth defects research.

Authors:  M R Bernfield
Journal:  Calif Med       Date:  1970-02

7.  Clinical and enzymatic variations in G M1 generalized gangliosidosis.

Authors:  H S Singer; I A Schafer
Journal:  Am J Hum Genet       Date:  1972-07       Impact factor: 11.025

8.  Ultrastructure of heart muscle in generalized gangliosidosis GM1.

Authors:  K P Backwinkel; D B von Bassewitz; L Diekmann; H Themann
Journal:  Z Kinderheilkd       Date:  1971

9.  The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism.

Authors:  M Cantz; J Gehler
Journal:  Hum Genet       Date:  1976-06-29       Impact factor: 4.132

10.  Hydrolysis of GM1-ganglioside by human liver beta-galactosidase isoenzymes.

Authors:  M W Ho; P Cheetham; D Robinson
Journal:  Biochem J       Date:  1973-10       Impact factor: 3.857
  10 in total

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