Prions Flashcards
1
Q
What is the variation in the prion problem?
What does this abnormality present?
A
- PrPc–> PrPsc
- Spongiform appearance and aggregation as amyloid-like plaques in the brain
2
Q
How do you get kuru?
How do you get Creutzfeldt-Jakob Disease?
A
Cannabilism
-90% sporadic, 10% familial - not from food
3
Q
What is the age group with CJD?
What is a key initial symptom?
A
- > 50 years old
- ataxia
4
Q
What is the onset of Gerstmann-Straussler-Scheinker (GSS) syndrome?
A
- ages 35-55 - inherited
5
Q
What is age of of onset of Fatal familial insomnia (FFI)?
A
- Genetic mutations, 20-70 yrs, - inherited
6
Q
What is the first mutation that must occur in codon 178 that must occur in a genetic mutation?
- What occurs if codon 129 the aa is mutated to valine?
- What occurs if codon 129 the aa is mutated to methionine?
A
- Aspartic acid –> asparagine
- the inherited form of CJD
- FFI
7
Q
What is the difference between traditional CJD and variant CJD? **
A
- CJD: age of onset >50, not really plaques
- vCJD: age of onset:19-24, extensive plaque formation.
8
Q
How is prion disease confirmed?
A
- by detecting proteinase K-resistant PrPsc in western blot on autopsy
9
Q
How was variant CJD formed?
A
- ingestion of beef contaminated with BSE
