Prions

Question 1

What causes prion disease

Answer 1

Caused by a misfolded PrPc that becomes a PrPsc protein
This is a cellular protein found on the outside of cell walls in mammals
it is most highly expressed in the CNS

Question 2

Properties of prion diseases

Answer 2

Neurological disorders
Neuronal loss
invariably fatal
very long incubation periods

Question 3

Prion Disease in sheep and goats

Answer 3

Scrapie, atypical scrapie

Question 4

Prion Disease in deer and elk

Answer 4

chronic wasting disease

Question 5

prion disease in cattle

Answer 5

Bovine Spongiform Encephalopathy (bse)
also seen in goats and exotic ungulates

Question 6

Prion Disease in cats

Answer 6

Feline Spongiform Encephalopathy

Question 7

how long is the incubation period for BSE

Answer 7

4-6 years

Question 8

how is BSE spread

Answer 8

parts of an infected cow can be eaten by other cows and people
If a cow gets BSE, it most likely ate the contaminated feed during its first year of life.

Question 9

Common clinical signs of a prion disease

Answer 9

Weight loss, subtle behavioural changes
e.g. isolated, flighty, lies down more, dull
Licking lips, grinding teeth, scratching
Pruritus (scrapie)
Posture, gait
Ataxia, tremors
None, animal found dead

Question 10

Prion secretion to the environment in different types of prion diseases

Answer 10

BSE- barely any prion is secreted into the environment
CWD- lots of prion is secreted into the environment so it spreads between animals and is harder to control as there are reservoirs in the environement