9- Prions disease

Question 1

What is a prion

Answer 1

misfolded infectious protein which transmits its shape onto normal cellular proteins

Question 2

What is the normal cellular prion protein called

Answer 2

PrPc

Question 3

What is the misfolded cellular prion protein called

Answer 3

PrPsc

Question 4

PrPsc accumulates in tissues and causes what

Answer 4

Neurological disorders
Neuronal loss
No cure

Question 5

Give an example of a prion disease in cows and sheep (2)

Answer 5

Cattle= bovine spongiform encephalopathy
Sheep= Scrapie, atypical scrapie

Question 6

Name 3 clinical signs of prion diseases

Answer 6

Weight loss
Subtle behaviour changes- isolated, flighty, lies down more, dull
Licking lips, grinding teeth, scratching
pruritus (scrapie)
gait, posture
Ataxia, tremors
Animal is dead

Question 7

What is the national scrapie plan

Answer 7

Breeding programme whichbreeds sheep with the PRNP gene- highly resistant to classical scrapie

Question 8

CWD stands for

Answer 8

Chronic wasting disease

Question 9

T/F animals with BSE don’t shed the prion

Answer 9

True

Question 10

T/F scrapie/CWD is shed in the environment

Answer 10

True

Question 11

Why do prion diseases have long incubation periods

Answer 11

because the PrPs needs to accumulate (not sure if this is right)

Question 12

What is atypical scrapie

Answer 12

is a spontaneous disease of older animals - It can occur in animals considered genetically resistant to classical scrapie