CH.10.16 Prions

Question 1

what is a prion?

Answer 1

the only known infectious protein

Question 2

how are prion proteins produced?

Answer 2

by neurons and believed to be involved in signaling

Question 3

how are the prion proteins amino acid encoded

and how is their folding arranged

Answer 3

by dna

alpha helix

Question 4

what happens to an abnormal prion?

Answer 4

misfolding
folding becomes beta sheet
they cant be broken down by proteases

Exposure to PrPsc causes abnormal folding of normal PrPc

Question 5

what happens when PrPsc accumulates?

and what about astrogliosis ?

Answer 5

causes nerve cell death and spongiform appearance of brain tissue
transmissible spongiform encephalopathies (TSE)

astrogliosis also known as amyloid plaques also results due to nerve cell death

Question 6

causes of TSE

Answer 6

it is spontaneous
genetic
Iatrogenic (5%): acquired through medical procedure
Consumption of infected meat (not quantified)

Question 7

Kuru (laughing sickness) means to ?

Answer 7

shake

was the only TSE in history to reach epidemic proportions in humans

Question 8

kuru afflicted tribe of ?

Answer 8

Papua New Guinea

Question 9

kuru studied by ___ and awarded nobel prize for ?

Answer 9

Gajdusek and Alpers

transmissibility

Question 10

kuru is transmitted by

Answer 10

cannibalistic rituals

Question 11

Bovine Spongiform Encephalopathy (BSE) affects who?

Answer 11

cattle

Question 12

prions were first thought to be __

Answer 12

viral

Question 13

what did Stanley B. Prusiner do?

Answer 13

identifies cause as an “infectious protein” and identified the link between BSE and vCJD

Question 14

prion came from the phrase?

Answer 14

proteinaceous infectious particle”

Question 15

Chronic Wasting Disease is found in ?

Answer 15

deer and elk