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

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

Mouse number	DPI	PrPSc IHC	PrPSc western blot	Clinical TSE suspect	Clinical notes (reason for euth) & relevant necropsy findings
B302–2	77^a	–	nt	No	normal
B299–1	77^a	–	nt	No	normal
B302–3	239^a	–	nt	No	normal
B302–4	239^a	–	nt	No	normal
B304–1	529^a	–	nt	No	normal
B304–2	529^a	–	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	782^a	–	nt	No	normal
B306–4	782^a	–	nt	No	normal
B340–1	784	–	–	No	urine scalding
B295–3	798	–	–	No	normal

^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

ISSN: 2051-5960