Prions - A.Prun

Question 1

Cannibalistic tribe in Papa New Guinea develops Kuru.

What were the strange symptoms?

Answer 1

Strange walk, slurred speech, facial tics, uncontrolled laughter, and certain death

Question 2

What is the conformation of prions that makes them so durn stable despite boiling, freezing, desiccation, UV, and chemicals?

Answer 2

Beta sheet form

Question 3

What is the infectious agent in prion diseases?

Answer 3

The PrP protein

Question 4

What does PrP-sc do?

Answer 4

It causes misfolding in normal PrP-c proteins into PrP-sc proteins and forms amyloid plaques.

Question 5

How do you diagnose Creutzfeldt-Jakob Disease?

Answer 5

EEG
MRI
CSF - elevated protein
Western Blot - PrP-sc
DNA sequence it
Brain Biopsy

Question 6

Compare and contrast Alzheimer’s disease and spontaneous Creutzfeldt-Jakob disease.

Answer 6

Same:

• Amyloid formation
• Dementia
• Generally older adults

Different

• Specific protein build-up is different
• Longer progression in Alz, CJD faster

Question 7

Effective treatments for prion disease?

Answer 7

nope, always fatal

Question 8

What is a potential prion disease therapy involving inhibition of the unfolded protein response?

Answer 8

eIF is generally phosphorylated in cases where misfolded proteins are detected in the ER -> leading to cell death

By inhibited the eIF response it could be possible to save cells from dying so much!