Skip to main content

Table 1 Transmission study of human, genetic mutant Y226X PrP isolate 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

B321–3

77a

nt

No

normal

B325–2

77a

nt

No

normal

B326–1

240a

nt

No

normal

B326–2

240a

nt

No

normal

B326–3

509a

nt

No

normal

B326–4

509a

nt

No

normal

B323–1

593

+

+

No

injury necessitating euthanasia

B322–1

601

+

+

Yes

urine scalding, ataxic, poor nest, thin, dilated, thickened uterus, consolidated lung lobe

B322–2

609

Yes

thin, circling, kyphosis, poor nesting, ataxic, tippy-toed gait

B323–2

680

Yes

thin, hunched, progressive paraparesis, bilaterally distended and inflamed uterus

B324–1

716

+

+

No

tremor, mild ataxia, abnormal respirations, good body condition

B323–3

718

+

+

Yes

Weight loss, poor coat quality, hunched posture

B326–5

720a

No

old age, thin

B324–2

762

Yes

thin, hunched, wobbly

  1. a- Indicates mice that were stereotactically microinjected into the striatum. As described in the methods, this technique was used to facilitate possible early detection of PrPSc replication at the site of the needle track, as was previously demonstrated [9]. 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 when they developed conditions requiring euthanasia for humane reasons. nt = not tested