Protein misfolding

Question 1

… is the normal form while … is the abnormal form of the prion protein.

Answer 1

PRPc, PRPsc(differ in 2,3 structure)…conformational change

Question 2

How does PRPsc replicate

Answer 2

It binds to PRPc and leads to flipping PRPc into the B conformation(PRPsc) and it can polymerize.

Question 3

… reverses formation of PRPsc

Answer 3

3M guanidine HCL

Question 4

PRPc is involved in…(5)

Answer 4

1) Sleep-wake cycle
2) Exploratory behavior
3) Memory recall
4) May cause neuronal cell death
5) loss of Purkinje cells(ataxia, gait)

Question 5

T/F: PRPsc alone can produce disease
Accumulation of PRPsc = …

Answer 5

False. Needs PRPc
Apoptosis