topic 3b - prions

Question 1

what does prion stand for?

Answer 1

proteinaceous infectious agents

Question 2

why are prions not considered living things?

Answer 2

they do not have any genetic material

Question 3

what is bovine spongiform encephalopathy (BSE)?

Answer 3

“mad cow disease”

Question 4

in “mad cow disease” _______ (fibrous masse of proteins) interfere with normal nerve cells to make brain ______

Answer 4

plaquesspongey

Question 5

how do cows infect humans w. mad cow disease?

Answer 5

eating meat from cows fed animals parts from sick animals that could not be given to humans

Question 6

in mad cow disease what is the normal occuring proteins (cPrP) shape and flexibility:

Answer 6

alpha helix

Question 7

mad cow disease: in disease, what is the protein (pPrp)

Answer 7

beta sheet - resist acid, digestive enzymes, get into blood, go through blood brain barrier and flatten the cPrP proteins

Question 8

who is at risk of mad cow disease?

Answer 8

only those born susceptibility at risk (<40% of humans)

Question 9

what happens during disease of mad cow disease

Answer 9

• cPrP made - pPrP encountered -> begins flattening cPrP proteins

Question 10

scrapie - disease in: - symptoms:

Answer 10

• in sheep- symptoms: jump off cliffs? - not drink water -scrape bodies raw by rubbing against fences - gradual loss of strength, inability to stand, erratic behavior

Question 11

what was the first human prion disease to be studied?

Answer 11

kuru

Question 12

kuru (“trembling disease”) - discovered by? - disease from/in- symptoms - transmission- common in?

Answer 12

• stanley pruisnew - fore tribe in New Guinea - symptoms: gradual loss of motor controlled death- transmission -> canibalism - common in: women and children (would eat brains of wise tribe members once they died)

Question 13

creutzfeldt-Jakob disease - found in?- caused by?

Answer 13

-heredity disease in 50-75 y/o -slow onset and death from degenerative brain function loss

Question 14

variant creutzfelt-Jakob-seen where?-humans get this not? how is it different from typical?

Answer 14

-Europe- humans get this not “mad cow disease”- different from typical: - younger people affected - no family history - linked to eating beef from BSE cows - linked to head injury

Question 15

chronic wasting disease - found where? - found in?- symptoms?

Answer 15

found where: north america found in: mule,deer, elk, some white tail deer, and some squirrels - symptoms: - loss of muscle tissue - nervous trembling - confusion, stumbling - not able to get nutrients from food - lose muscle tone and your body begins to digest itself