Test 2: prions

Question 1

— are RARE progressive neurodegenerative disorders affecting animals and humans

Answer 1

transmissible spongiform encephalopathies (TSEs)

Question 2

TSEs cause

Answer 2

Spongiform degeneration, neuronal loss, and astrocytic gliosis

Question 3

large holes in brain caused by TSEs are

Answer 3

spongiform degeneration

Question 4

—- is a proliferation of astrocytes in damaged areas of the CNS that leads to a scar

Answer 4

Astrocytic gliosis

Question 5

TSEs have a long or short incubation time?

Answer 5

long- really long

Question 6

can a human survive a TSEs

Answer 6

no- invariably fatal

Question 7

increased incubation time when transfered to a new host indicates TSEs have

Answer 7

species barrier

Question 8

TSEs are caused by

Answer 8

prion- misfolded protein
no DNA/no RNA

Question 9

three routes of transmission for prion disease

Answer 9

sporadic - mutation
familial- hereditary
acquired- foodborne, bloodborne, iatrogenic

Question 10

Kuru causes

Answer 10

shiver, trembling, smiling disease
unstead gai, tremor, slurred speech

foodborne disease from ritual cannibalism

Question 11

Kuru was probably caused by

Answer 11

sporadic mutation( classical creutzfeldt-jakob disease (CJD)

then foodborne from eating infected host

very long incubation period >50 years

Fore people in papua new guinea

Question 12

CJD is usually caused by sporadic mutation but can arise from —-

Answer 12

iatrogenically (medical procedure)

infected equipment, human growth hormone from infected person

classical creutzfeldt-jakob disease

Question 13

scrapie effect what animals

Answer 13

sheep and goats

Question 14

scrapie presents with what symptoms

Answer 14

tremor
itchiness(pruritus), wool loss, paralysis and death

sheep and goat

Question 15

what countries are scrapie free

Answer 15

australia and new zealand

Question 16

how is scrapie spread

Answer 16

oral or via wounds in contaiminated pasture or by contact

Question 17

scrapie does not cause

Answer 17

inflammatory reaction

can not use serology to detect

Question 18

prion causes spongiforms by

Answer 18

misfolded protein fibrils group in the CNS
cause amyloid plaques
disrupt normal stucture- vacuole formation in the neurons
cause holes in brain tissue

Question 19

prions are resistant to

Answer 19

resistant to heat, nucleases, Xrays and UV

Question 20

what is the structure of prion protein

Answer 20

normal is alpha helical

abnormal has more beta sheets making it resistant to heat, nucleases, xrays and UV

Question 21

prions are partially resistant to —

Answer 21

protease

Question 22

how to test for prion

Answer 22

post mortem histopath of the obex part of the brain
immunohistochemistry
wetesern blot with monocolonal antibodies

some tests can detect BSE 2-3 months before clinical signs

Question 23

PMCA can detect — but can’t detect —

Answer 23

Protein Misfolding Cyclic Amplification

used to find vCJD using peripheral tissues but unable to detect sporadic CJD

Question 24

what is the normal function of prion proteins?

Answer 24

no one really knows

regulate cell death, long term memory, stem cell renewal, innate immunity?

Question 25

BSE causes ---

Answer 25

nervousness, skin tremors, manic kicking during milking, loss of condition, death in a few weeks | mad cow disease Bovine spongiform encephalopathy

Question 25

BSE causes ---

Answer 25

nervousness, skin tremors, manic kicking during milking, loss of condition, death in a few weeks | mad cow disease Bovine spongiform encephalopathy

Question 26

what caused BSE

Answer 26

contaminated feed (meat and bone meal) spontaneous mutation

Question 27

what is meat and bone meal

Answer 27

animal waste the is rendered into a protein rich feed

Question 28

old or young people are more susceptibile to prion

Answer 28

young- age dependent

Question 29

three types of BSE prion

Answer 29

c-type: classical Ltype and H type: atypical

Question 30

Studies with mice suggest that H-type BSE may shift its disease phenotype to that of----type BSE upon transmission

Answer 30

C (classical) H type occurs through sporadic mutation, tranmission through ingestion, shift to C-type inside host, changes are preserved through successive transmissions

Question 31

average age for sporatic vs variableCJD

Answer 31

variable- 26 (survive for a 12 months) sporadic- 67 (survive for 4 months)

Question 32

lesion profiles are the same for ---- but different for

Answer 32

vCJD and BSE scrapies and sCJD

Question 33

transmissible mink encephalopathy was probably caused by

Answer 33

eating BSE infected cow

Question 34

chronic wasting disease infect ---

Answer 34

deer and elk

Question 35

chronic wasting disease is spread by

Answer 35

stays in environment so maybe by shedding from digestive tract, most likely via saliva and feces can be found in muscle of deer- no person transfer, yet present in farm and wild deer and elk

Question 36

Asymptomatic deer excrete infectious prions in feces, spreading what disease

Answer 36

Chronic wasting disease (horizontal transmission, fecal oral route, live in soil)