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

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.
AuthorsM C MacBrinn, S Okada, M W Ho, C C Hu, J S O'Brien
JournalScience (New York, N.Y.) (Science) Vol. 163 Issue 3870 Pg. 946-7 (Feb 28 1969) ISSN: 0036-8075 [Print] United States
PMID4236799 (Publication Type: Journal Article)
Chemical References
  • Glycoproteins
  • Glycosaminoglycans
  • Galactosidases
  • Galactose
Topics
  • Carbohydrate Metabolism, Inborn Errors (metabolism)
  • Galactose (metabolism)
  • Galactosidases (metabolism)
  • Glycoproteins (metabolism)
  • Glycosaminoglycans (metabolism)
  • Humans

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