Correction of β-glucuronidase deficiency in mice by intrasplenic transplantation of isolated hepatocytes

Y. Ilan*, D. Shouval, E. Galun, R. Feder, R. Adler, S. Yatziv

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Correction of enzyme activity was successfully accomplished in β-glucuronidase deficient C3H/HeJ mice after intrasplenic transplantation of isolated hepatocytes obtained from normal Balb/C donors mice. Enzyme activity increased from a mean of 1.1 ± 0.2 nmol/h/ml on day 0 to 15.8 ± 3.3 nmol/h/ml on day 55 post-transplantation. Examination of enzyme activity in liver tissue by repeated liver biopsies revealed an increase in β-glucuronidase activity from a mean of 1.85 ± 0.6 nmol/h/mg protein on day 0 up to 42.6 ± 7.3 nmol/h/mg protein on day 35 post-transplantation. β-Galactosidase and β-N-acetyl-glucosaminidase activities were not significantly changed throughout the study. Intrasplenic transplantation of hepatocytes isolated from β-glucuronidase-deficient mice did not induce a significant change in three examined hydrolases. These results suggest that transplantation of isolated hepatocytes may be used as an effective tool for therapy of lysosomal enzyme deficiency in mice.

Original languageAmerican English
Pages (from-to)97-104
Number of pages8
JournalInternational Hepatology Communications
Issue number2
StatePublished - Feb 1995
Externally publishedYes


  • Hepatocyte transplantation
  • β-Glucuronidase


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