These mice carry an allele that confers a deficiency of beta-glucuronidase (beta-D-glucuronoside glucuronosohydrolase) and a transgene carrying the human beta-glucuronidase gene. Whereas the Gusbmps allele produces virtually no murine beta-glucuronidase, these mice with the human beta-glucuronidase gene express high levels of human beta-glucuronidase activity in all tissues examined and are phenotypically normal. . Biochemically, both the intralysosomal storage of glycosaminoglycans and the secondary elevation of other acid hydrolases are corrected.

These mice carry a spontaneous mutation at the Gusb locus, that by itself causes a deficiency of beta-glucuronidase, and a transgene carrying the human beta-glucuronidase gene, that corrects the beta-glucuronidase deficiency. These mice are useful in studies relating to mucopolysaccharidosis VII (Sly syndrome) in humans.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

<a target="_blank" href="https://www.jax.org/jax-mice-and-services/customer-support" rel="noreferrer">Contact Customer Service</a> for more information.