Lecture 10 - Prions

Question 1

What are TSEs?

Answer 1

Transmissible Spongiform Encephalopathies (TSEs)

Question 2

What type of disease is TSE?

Answer 2

Neurodegenerative disease leading to death

Question 3

Prions lack what?

Answer 3

A genome

Question 4

How can TSE be diagnosed?

Answer 4

Only by autopsy

Question 5

Scrapie is apparent in what species?

Answer 5

Sheep and goats, not in humans!

Question 6

Prions are…

Answer 6

Protein + Infection located on the surface of neurons

Question 7

PrPc

Answer 7

Cellular prion protein that is highly conserved in animals and is non-infectious

Question 8

PrPSc

Answer 8

Infected form of cellular prior protein for scrapie.

Question 9

PrPSc lacks what…

Answer 9

Nucleic acids, but is still infectous

Question 10

PrPSc is derived from?

Answer 10

PrPc

Question 11

3 Differences between PrPc and PrPSc

Answer 11

PrPc - alpha helices, soluble, not sticky.

PrPSc - beta-sheets, insoluble, sticky

Question 12

How is PrPSc synthesized?

Answer 12

PrPc is made in the nucleus and brought to the cell surface. PrPSc from another cell comes and alters PrPc to form PrPSc. PrPSc is taken in by endocytosis and is accumulated in cell causing cell death

Question 13

How does the cell break down PrPc

Answer 13

By proteases, Sc is resistant to these!!

Question 14

Where is PrPc most commonly found in the body?

Answer 14

Brain

Question 15

Better name for Bovine Spongiform enecephalopathy

Answer 15

MAD COW

Question 16

How is mad cow disease occurring

Answer 16

Cows being fed other cows remains, and the humans eating those infected cows

Question 17

Three types of CJD (Creutzfeldt-Jakob Disease)

Answer 17

sporadic, hereditary and latrogenic

Question 18

Kuru (symptoms, transmission)

Answer 18

Body tremors and obtained from cannabilism