Which of the following are accurate characteristics of prions? Select all that apply

Question

A) Disease usually progresses slowly.
B) The primary manifestation includes ataxia and dementia.
C) Prions are protease sensitive.
D) Prions tend to aggregate into amyloid-like plaques in the brain.
E) Prions lack reproductive functions so are not very harmful to humans.

Answer 1

Ans: A, B, D
Feedback:
The various prion-associated diseases produce very similar pathologic processes and symptoms in the hosts and are collectively called transmissible neurodegenerative diseases. All are characterized by a slowly progressive, noninflammatory neuronal degeneration, leading to loss of coordination (ataxia), dementia, and death over a period ranging from months to years. Prion proteins in disease (called PrPSC) are resistant to the action of proteases (enzymes that degrade excess or deformed proteins). It is believed that PrPSC binds to the normal PrPC on the cell surface, causing it to be processed into PrPSC, which is released from the cell and then aggregates into amyloid-like plaques in the brain.