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Flashcards in Prions Deck (9)
1

Prion disease - mechanism

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).

2

Prion disease is transmissible via (generally)

CNS related tissue

3

Prion disease - PrP (sc) characteristics

resistant to standard sterilizing procedures, including standard autoclaving

4

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

- spongiform encephalopathy
- dementia
- ataxia
- death

5

Prion disease - types

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

6

Prion disease - Kuru

aquired prion disease note in tribal populations practing human cannibalism

7

Prion disease - Bovine spongiform encephalopathy

AKA "mad cow disease"

8

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

via CNS related tissue:
1. Creutzfeldt-Jakob disease --> iatrogenic
2. Bovine spongiform encephalopathy --> food contamination
3. Kuru --> cannibalism

9

Prion disease - types

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