Prions

Question 1

what does Kuru mean

Answer 1

trembling from fear and cold

Question 2

what is the clinical course for Kuru

Answer 2

prolonged headache and minor loss of coordination. symptoms rapidly progressed and by 4 months victims unable to walk requiring constant care. Speech became disturbed and completely disappeared. Victims lost ability to swallow. Death occurred within 1 year of onset of symptoms

Question 3

When did Kuru first appear

Answer 3

~1900

Question 4

Kuru was a disease primarily of who

Answer 4

women and children

Question 5

What was the name of the tribe of people who experienced Kuru

Answer 5

Fore in New Guinea

Question 6

What seemed to be the most reasonable cause of Kuru

Answer 6

Cannibalism

Question 7

Why was cannibalism practiced in the New Guinea tribe

Answer 7

Was a religious ceremony in which the women would eat the flesh of the dead, mainly the brain.

Question 8

How did the New Guinea religious ceremony start

Answer 8

The men ate all the food and the women were starving, so they begin to eat the dead to get protein and disguised it as a religious ceremony

Question 9

What characteristics about the brains taken resembled another disease

Answer 9

loss of neurons (spongiform encephalopathy) and activation of astrocytes

Question 10

Which disease did the Kuru brains resemble

Answer 10

Creutzfeldt-Jakob disease

Question 11

What is Creutzfeldt-Jakob disease also known as and why

Answer 11

Spongiform encephalopathy due to the gaping holes where the neurons had once been

Question 12

What test animals was chosen to be inoculated with the Kuru brain

Answer 12

Rhesus monkeys and Chimpanzees

Question 13

How many people were dying of Kuru in the Fore tribe

Answer 13

200 persons each year (1% of tribe)

Question 14

What was the ratio of men to women

Answer 14

3:1

Question 15

The women used ____ to prepare the tissues and packed the brain in small ____ to be steamed over an open fire

Answer 15

Bamboo blades; bamboo tubes

Question 16

When NIH experiments and duplicates the preparation conditions what did they find

Answer 16

10 million infectious agents per gram of steamed brain

Question 17

What impact on medicine, neurology, and infectious disease did Kuru have

Answer 17

opened new area of slow virus diseases, opened new area of transmissible spongiform encephalopathy, and lead to identification of new infectious agent ,the prion

Question 18

Viruses are considered ____ agents and prions are considered ____ agents

Answer 18

Conventional; unconventional

Question 19

Subacute Sclerosing Panencephalitis is caused by what

Answer 19

a measles virus with defective Matrix protein

Question 20

What is the incubation period of SSP

Answer 20

7-10 days

Question 21

What is the clinical progression of SSP

Answer 21

Progressive dementia , ataxia, and myoclonus. Death usually occurs within 1-3 years

Question 22

What are some slow virus diseases found in animals

Answer 22

Scrape in sheep; Transmissible mink encephalopathy; Bovine spongiform encephalopathy

Question 23

What are some slow virus diseases found in humans

Answer 23

Creutzfeldt-Jakob disease; Variant Creutzfeldt-Jakob disease (mad cow); Kuru (but kuru is extinct)

Question 24

What is the prion hypothesis

Answer 24

the prion is a small proteinacious infectious particle which is resistant to inactivation by most procedures that modify nucleic acids

Question 25

This protein was designated ____ and postulated to be a protein that is infectious

Answer 25

PrP (Proteinase-resistant protein)

Question 26

Describe PrP

Answer 26

smaller than conventional viruses, remarkable hydrophobic properties, resistant to radiation and UV light, formaldehyde, alcohols, proteinase K, autoclaving and nucleases

Question 27

what are the two isoforms of PrP

Answer 27

PrPc and PrPsc

Question 28

Which isoform is PrP is the normal cellular form

Answer 28

PrPc

Question 29

Which isoform of PrP is the abnormal form that causes prion protein illness

Answer 29

PrPsc

Question 30

What makes the two isoforms have such different physical characteristics

Answer 30

PrPc is primarily folded in alpha helix and PrPsc is folded in Beta sheets

Question 31

What are the 3 forms of CJD

Answer 31

Sporadic, Familial, and Iatrogenic

Question 32

which form of CJD occurs in ages b/t 50-79 and is 85% of all cases

Answer 32

Sporadic

Question 33

which form of CJD occurs in ages less than 50 years of age

Answer 33

Familial

Question 34

which form of CJD accounts for 1% of all cases, has a median incubation period of 13 years, and is believed to be Kuru

Answer 34

Iatrogenic

Question 35

What is vCJD

Answer 35

variant form CJD, and is really mad cow in humans

Question 36

what age group does vCJD occur in

Answer 36

26 years of age and death within 13 months of symptoms