Prions

Question 1

Where are normal prion proteins found?

Answer 1

Brain

Question 2

Abnormally folded prion proteins are?

Answer 2

NOT broken down

Question 3

Abnormally folded prion proteins are not broken down. What does this cause?

Answer 3

They accumulate = disease

Question 4

PRPc

Answer 4

Normal prion proteins in the brain

Question 5

PRPsc

Answer 5

Abnormal prion proteins accumulating in the brain

Question 6

What changes are seen in the brain with accumulation of abnormal prion proteins?

Answer 6

Neuropil develops spongiform change (small vacuoles)

Question 7

Spongiform change

Answer 7

Small vacuoles

Question 8

Small vacuoles in the neuropil of the brain suggests?

Answer 8

Accumulation of abnormally folded prion proteins

Question 9

What symptoms do all abnormally folded prion proteins cause?

Answer 9

Dementia, psych problems and are fatal

Question 10

2 ways to get Creutzfeldt-Jakob Disease?

Answer 10

• Reused neurosurgical tools

- Eating Bovine (cow) meat after they were fed dead animal remains

Question 11

2 ways to get Creutzfeldt-Jakob Disease?

Answer 11

• Reused neurosurgical tools

- Eating Bovine (cow) meat after they were fed dead animal remains

Question 12

How do you get Kuru?

Answer 12

Canabalism of dead

Question 13

Extra symptoms with Kuru?

Answer 13

Shaking and ataxia

Question 14

How do you get Fatal Familial Insomnia?

Answer 14

Sporadic mutation

Question 15

Extra symptoms Fatal Familial Insomnia?

Answer 15

Progressive Insomnia

Question 16

What brain changes are seen with all prion diseases?

Answer 16

Brain atrophy (shrinkage)

Question 17

Symptoms of abnormal accumulation of misfolded prion proteins?

Answer 17

Brain Atrophy
Dementia
Muscle spasms
Psych problems
Death

Question 18

3 diseases caused by misfolded prion proteins?

Answer 18

Creutzfeldt-Jakob Disease
Kuru
Fatal Familial Insomnia