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Table 3 Transmission study of human, genetic mutant Q227X PrP to tg66 transgenic mice expressing human PrP

From: Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein

Mouse number DPI PrPSc IHC PrPSc western
Clinical TSE suspect Clinical notes (reason for euth) & relevant necropsy findings
B302–2 77a nt No normal
B299–1 77a nt No normal
B302–3 239a nt No normal
B302–4 239a nt No normal
B304–1 529a nt No normal
B304–2 529a nt No normal
B630–1 545 No lung neoplasia
B296–1 650 No eye neoplasia
B295–1 696 No injured, thin, ataxic, adequate nesting, lymphoma
B296–2 716 No injured, thin, barrel rolled twice, aware and responsive
B295–2 743 No distended abdomen, liver neoplasia
B296–3 756 No thin
B306–3 782a nt No normal
B306–4 782a nt No normal
B340–1 784 No urine scalding
B295–3 798 No normal
  1. a- Indicates mice that were stereotactically microinjected into the striatum. Mice injected using this technique were euthanized electively and tissues were processed to directly screen the injection needle track and adjacent brain by IHC for any PrP replication. Mice without asterisks were intracerebrally inoculated with a 30ul volume of brain homogenate and euthanized when they developed neurologic signs consistent with prion infection (none) or conditions requiring euthanasia for humane reasons or at the termination of the experiment at 782 and 798 dpi