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eagle-i ID


Resource Type

  1. Mus musculus


  1. Resource Description
    These mice (first described by the group of Dr. William S. Sly) constitutively express high levels of human GUSB. The transgene is expressed on the MPS VII mouse background, so only human GUSB enzyme is produced. "Male pronuclei of both F<sub>1</sub> and F<sub>2</sub> zygotes were microinjected with the <i>Eco</i>RV fragment of the human beta-glucuronidase cosmid clone. The F<sub>1</sub> zygotes were derived from C57BL/6J x LT/Sv crosses, and the F<sub>2</sub> zygotes were derived from an intercross of C57BL/6J x SJL/J F<sub>1</sub> hybrids. The human <i>GUSB</i> was placed onto the MPSVII mouse genetic background by mating male mice heterozygous for the human transgene with female mice homozygous for the <i>gus<sup>mps</sup></i> mutation. Tg<i>GUSB</i>/+,<i>gus<sup>mps/+</sup></i> were then mated to offspring without the human transgene to generate Tg-<i>GUSB</i>/+,<i>gus<sup>mps</sup>/gus<sup>mps</sup></i>." "The transgene contains an estimated 2 to 4 tandem repeat copies of 1.6 kilobases (kb) of the 5′ flanking sequence, the entire structural gene, and 3.8 kb of the 3′ flanking sequence of the human genomic GUSB gene. Thus, the TG mice produce no murine GUSB protein but maintain a normal phenotype because they produce 10 to 20 times higher than normal levels of human GUSB. They have been interbred to homozygosity and are maintained as a recombinant inbred strain."
  2. Related Publication or Documentation
    In utero transplantation of fetal liver cells in the mucopolysaccharidosis type VII mouse results in low-level chimerism, but overexpression of beta-glucuronidase can delay onset of clinical signs
  3. Related Publication or Documentation
    Correction of murine mucopolysaccharidosis VII by a human beta-glucuronidase transgene
  4. Genetic Alteration(s)
  5. Genetic Alteration(s)
  6. Phenotype Findings
    Normal phenotype
  7. Location
    Wolfe Laboratory
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Copyright © 2016 by the President and Fellows of Harvard College
The eagle-i Consortium is supported by NIH Grant #5U24RR029825-02 / Copyright 2016