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Table 4 Transmission study of human, genetic mutant G131V 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 blot Clinical TSE suspect Clinical notes (reason for euth) & relevant necropsy findings
B300–3 79a nt No normal
B339–3 79a nt No normal
B307–1 241a nt No normal
B307–2 241a nt No normal
B308–3 531a + nt No normal
B328–1 531a + nt No normal
B633–1 596 + No normal
B297–1 650 + No weight loss, hind limb weakness
B297–2 731 + Yes head bob, tremors, thin
B298–1 731 + Yes weak hind legs, thin, kyphosis, weepy eye, ataxia for 1 month
B297–3 735 + Yes thin, slight wobble, rough coat, squinty eyes, decent nest
B297–4 735 + Yes thin, slight wobble, rough coat, squinty eyes, decent nest
B632–1 758 + Yes thin, rough coat, mild ataxia
B298–2 769 + Yes thin, ataxic
B303–1 784 + Yes urine scalding, weak hind legs, tremor
  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 or conditions requiring euthanasia for humane reasons