| 摘要 |
<p>The present invention comprises non-human transgenic animals with: 1) a genome artificially altered to decrease or ablate endogenous PrP activity; and 2) proper health and development maintained through the administration of copper. The transgenic animal may have an endogenous gene altered to decrease endogenous PrP expression; ablated to eliminate endogenous PrP expression; or modified to express an exogenous form of PrP. The exogenous form of PrP includes a PrP gene from a genetically diverse species, a chimeric PrP gene containing sequences of the endogenous PrP gene from the PrP gene of a genetically diverse species; a mutated, deleted or otherwise altered form of the endogenous PrP, and PrP genes operably linked to an inducible promoter. Transgenic mouse of the invention are useful bioassay with their sensitivity to prion infection enhanced via the administration of controlled amounts of copper. Transgenic farm animals (e.g., cows) are useful for normal purposes with the benefit of not being susceptible to prions that normally infect animals of that species (e.g., BoPrPSc).</p> |
