Uptake and distribution of placental glucocerebrosidase in rat hepatic cells and effects of sequential deglycosylation

F. Scott Furbish, Clifford J. Steer, Nancy L. Krett, John A. Barranger

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157 Scopus citations


The clearance of native human placental glucocerebrosidase by rat liver shows the presence of two distinct enzyme forms with different recognition characteristics. The clearance and uptake of native enzyme by liver cells was compared to that of glucocerebrosidase sequentially treated with neuraminidase, β-galactosidase and β-N-acetylglucosaminidase. The initial rate of clearance of infused enzyme was increased greater than 10-fold for the asialo-, agalacto- and ahexoenzymes over that of native glucocerebrosidase. Incorporation of asialo enzyme was increased in hepatocytes over that of native enzyme, while the distribution of agalacto- and ahexoenzyme preparations was increased in non-parenchymal cells. This observation is consistent with the identification of a galactose receptor on hepatocytes and N-acetylglucosamine/mannose receptors on Kupffer cells. These data and inhibition studies by specified monosaccharide-terminal glycoprotein derivatives demonstrate the importance of these sugars in the uptake of this lysosomal enzyme by receptor-mediated endocytosis. Modification of the enzyme to expose certain monosaccharide moieties results in increased delivery to specific cell types. Therefore, naturally occurring receptors can be utilized for targeting glucocerebrosidase to the non-parenchymal cell in liver.

Original languageEnglish (US)
Pages (from-to)425-434
Number of pages10
JournalBBA - General Subjects
Issue numberC
StatePublished - 1981

Bibliographical note

Funding Information:
The authors wish to thank Dr. Roscoe O. Brady for his support and encouragement of the project and the National Lipid Disease Foundation for support of Ms. Nancy L. Krett.


  • Deglycosylation
  • Endocrytosis
  • Enzyme clearance
  • Glucocerebrosidase


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