Prion Disease and ALS

Question 1

What prion disease and in what animal was first to be identified?

Answer 1

Scrapie in sheep.

Question 2

What is the precursor protein of prions?

Answer 2

Prion protein, a cell-surface glycoprotein.

Question 3

What is the function of prion protein, a cell-surface glycoprotein?

Answer 3

Not certain, is copper binding

Question 4

What is the major secondary structure of prion protein?

Answer 4

Alpha-helical

Question 5

What is the structure of prion protein?

Answer 5

Alpha helical globular structure and random-coil hydrophobic structure with some glutamine repeats at the N-terminus.

Question 6

What are the two states that prion protein can be in and which is susceptible to protease digestion?

Answer 6

Scrapie and cellular (susceptible)

Question 7

What part of the prion protein (PrP) is necessary for disease?

Answer 7

Anchor

Question 8

Is hamster-mouse transmission of prion disease possible?

Answer 8

No

Question 9

Repeats of which two amino acids at the N terminus of PrP are important for disease?

Answer 9

Glutamine and asparagine

Question 10

What is the function of protein/RNA aggregation in a normal cell?

Answer 10

Overcoming stress. The aggregates are disassociated by heat shock proteins.

Question 11

What neurons are most affected by protein aggregates in ALS?

Answer 11

Spinal motor proteins