Prions Flashcards
What are prion caused diseases called?
Transmissible Spongiform Encephalopathies
What are prions made of?
Only misfolded proteins. No nucleic acids.
Why are prions so hard to control?
Extremely resistant to usual sterilization techniques.
What is the name of the normal protein involved in many prion diseases? The abnormal variant? Which is soluble?
PRP-C and PRP-Sc.
PRP-C
Why is there no immune response to PRP-C
They are naturally occurring proteins
What does the Sc in PRP-Sc stand for?
Scrapie
What layer of structure is different for PRP-Sc?
secondary. The primary is the same, but the secondary structure is dominated by beta conformation
Why is PRP-Sc a problem?
It is almost completely insoluble and highly resistant to protease digestion.
How does a prion disease spread in a brain?
When PRP-Sc comes into contact with PRP-C it converts it into more of itself. Later amyloid fibers can also spread through seeding.
What is CJD?
A degenerative brain disorder leading to dementia and death.
How is CJD distinguished from Alz.?
Progression occurs over about 4.5 months, death within a year.
Is CJD typically sporadic or inherited?
Sporadic
How is vCJD distinguished from CJD?
vCJD typically affects 28-29 year olds. CJD typically influences people over 50.
vCJD is also linked to exposure, often through food.
Where is Kuru from?
New Guinea
How is Kuru spread?
Endocannibalistic activity
