Enzyme therapy IV: A method for determining the In Vivo fate of bovine β-glucuronidase in β-glucuronidase deficient mice

S. R. Thorpe, M. B. Fiddler, R. J. Desnick

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

Abstract

A mammalian model system was developed to determine the in vivo fate of intravenously administered bovine β-glucuronidase in β-glucuronidase deficient mice. Sensitive and reliable discrimination of bovine liver β-glucuronidase activity from residual murine tissue activities was achieved by selective heat inactivation of the bovine activity, providing the ability to monitor the fate of administered enzyme. Following injection, the bovine activity was rapidly cleared from the circulation and recovered almost exclusively in the liver. Subcellular fractionation of hepatic tissue obtained 2 hours post injection localized 72% of recovered activity in the lysosomally-enriched fraction. This mammalian system provides an in vivo model to serially evaluate and maximize methods for the protection and delivery of an exogenous enzyme, prior to trials of enzyme replacement in patients with inherited enzymatic deficiency diseases.

Original languageEnglish
Pages (from-to)1464-1470
Number of pages7
JournalBiochemical and Biophysical Research Communications
Volume61
Issue number4
DOIs
StatePublished - 23 Dec 1974
Externally publishedYes

Fingerprint

Dive into the research topics of 'Enzyme therapy IV: A method for determining the In Vivo fate of bovine β-glucuronidase in β-glucuronidase deficient mice'. Together they form a unique fingerprint.

Cite this