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Biochemical Genetics

, Volume 16, Issue 11–12, pp 1079–1084 | Cite as

Glycosaminoglycan accumulation with partial deficiency of β-glucuronidase in the C3H strain of mice

  • Shaul Yatziv
  • Robert P. Erickson
  • Robert Sandman
  • William van B. Robertson
Article

Abstract

Young (60–80 days) mice of the low β-glucuronidase strain, C3H/HeJ, showed no differences in hepatic levels of glycosaminoglycans (GAGs) when compared to the randombred, “normal” Swiss-Webster mice of the same age. However, by 12 months of age hepatic GAG is nearly twice as high in C3H/HeJ mice as in Swiss-Webster mice. Studies of β-glucuronidase, β-galactosidase, and N-acetyl-β-glucosaminidase in four tissues of the two types of mice at the two ages revealed that glucuronidase was the only enzyme with lower activity in the C3H/HeJ strain.

Key words

β-glucuronidase lysosomal enzymes mucopolysaccharidosis glycosaminoglycans mice 

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Copyright information

© Plenum Publishing Corporation 1978

Authors and Affiliations

  • Shaul Yatziv
    • 1
  • Robert P. Erickson
    • 1
  • Robert Sandman
    • 1
  • William van B. Robertson
    • 2
  1. 1.Department of PediatricsUniversity of CaliforniaSan Francisco
  2. 2.Children's Hospital at Stanford, Department of PediatricsStanford University School of MedicineStanford

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