Prions Flashcards
What is the most common human prion disease?
CJD
What are the causes of CJD?
1- sporadic
2- familial
3- Iatrogenic
What are the signs and symptoms of sporadic CJD?
1- spongiform encephalopathy
2- RAPID loss of brain function
3- mental deterioration
What causes prion disease?
No virus particles, bacteria or fungi associated with the condition.
No nucleic acid is associated with infectivity.
What is the immune response to prion infection?
There is no immune response
Explain the protein only hypothesis:
Prions are encoded by the host
PrPc misfolds and converts to PrPsc
The misfolding arises via mutation
PrPsc causes more PrPsc to be make
Who can PrPsc be transmitted to?
An animal that encodes PrPc only
What is the normal function of PrPc?
Maintaining the brain white matter, regulating the innitation of immune cells, has responses to oxidative stress, and is involved in neuron formation
How many types of PrPsc are there?
4
Which forms of PrPsc are associated with sCJD? vCJD?
sCJD= type 1
vCJD=type 4
What do accumulations of PrPsc become?
amyloid fibers
How is sCJD diagnosed?
1- Brain biopsy (spongiform change)
2- CSF
3- MRI and EEG changes
How is sCJD treated?
There is no treatment
Which population si sCJD more prevalent in?
older populations
How do humans contract vCJD?
Transmission of BSE (cows to humans)
