Prions

Question 1

Prion disease - mechanism

Answer 1

it is caused by conversion of a normal (predominantly α-helical) protein termed prion protein PrP (c) to a β-pleated form, PrP (sc)
PrP (sc) resists protease degradation and facilitates the conversion of still more PrP(c) to (sc).

Question 2

Prion disease is transmissible via (generally)

Answer 2

CNS related tissue

Question 3

Prion disease - PrP (sc) characteristics

Answer 3

resistant to standard sterilizing procedures, including standard autoclaving

Question 4

Prion disease - accumulation of PrP (sc) results in (clinically)

Answer 4

• spongiform encephalopathy
• dementia
• ataxia
• death

Question 5

Prion disease - types

Answer 5

1. Creutzfeldt-Jakob disease
2. Bovine spongiform encephalopathy
3. Kuru

Question 6

Prion disease - Kuru

Answer 6

aquired prion disease note in tribal populations practing human cannibalism

Question 7

Prion disease - Bovine spongiform encephalopathy

Answer 7

AKA “mad cow disease”

Question 8

Prion disease is transmissible via - (specificly for every type)

Answer 8

via CNS related tissue:

1. Creutzfeldt-Jakob disease –> iatrogenic
2. Bovine spongiform encephalopathy –> food contamination
3. Kuru –> cannibalism

Question 9

Prion disease - types

Answer 9

1. Creutzfeldt-Jakob disease
2. Bovine spongiform encephalopathy
3. Kuru