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Enzyme replacement with recombinant beta-glucuronidase in the newborn mucopolysaccharidosis type VII mouse.

Abstract
beta-Glucuronidase injected i.v. into newborn mucopolysaccharidosis VII mice was cleared from the circulation in less than 1 h and taken up by tissues in a distribution corresponding to the location of the mannose 6-phosphate receptor. One h after a 3.5-mg/kg beta-glucuronidase injection, beta-glucuronidase levels were equal to or greater than normal in every organ examined with the exception of the brain, where 31% normal activity was present. Enzyme was detectable histochemically in the major sites of pathology for mucopolysaccharidosis VII including bone, brain, heart, and fixed tissue macrophages. The half-life of recombinant beta-glucuronidase activity in various organs of injected mucopolysaccharidosis VII mice was 1.5 to 4.5 d. These studies show that recombinant beta-glucuronidase administered to newborn mice reaches the sites of clinically important storage in murine mucopolysaccharidosis VII.
AuthorsC Vogler, M Sands, A Higgins, B Levy, J Grubb, E H Birkenmeier, W S Sly
JournalPediatric research (Pediatr Res) Vol. 34 Issue 6 Pg. 837-40 (Dec 1993) ISSN: 0031-3998 [Print] United States
PMID8108204 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Recombinant Proteins
  • Glucuronidase
Topics
  • Animals
  • Animals, Newborn
  • Glucuronidase (administration & dosage, pharmacokinetics, therapeutic use)
  • Half-Life
  • Histocytochemistry
  • Injections, Intravenous
  • Mice
  • Mice, Mutant Strains
  • Mucopolysaccharidosis VII (drug therapy, enzymology, genetics)
  • Recombinant Proteins (administration & dosage, pharmacokinetics, therapeutic use)
  • Tissue Distribution

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