Prions Flashcards
1
Q
Define prions
A
Small proteinaceous infectious particles that are resistant to processes that normally cause protein denaturing
2
Q
Characteristics of prions
A
Made of proteins
Don’t cause immunogenicity
Resistant to Thactivation
Reproduce by causing normal protein to fold to scrapie protein
causes vacuolation → spongiform
3
Q
Examples of human prionoses
A
FFI
kuru
Creutzfeldt Jacob Disease (CDJ)
Variant (CDJ)
Gerstmann- Straussler- SCheinker Syndrome
4
Q
Pathogenesis of prions
A
PRNP gene on chromosome 20
Normal protein → Abnormal Protein
Disassociates
Plaques form between neurons
Internalization of plagues → spongiform
5
Q
Clinical Manifestations
A
Cognitive impairment
Ataxia
Myoclonic Jerks
Mutism
FFI
