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Fall 22: micro > Test 2: prions > Flashcards

Test 2: prions Flashcards

1
Q

— are RARE progressive neurodegenerative disorders affecting animals and humans

A

transmissible spongiform encephalopathies (TSEs)

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2
Q

TSEs cause

A

Spongiform degeneration, neuronal loss, and astrocytic gliosis

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3
Q

large holes in brain caused by TSEs are

A

spongiform degeneration

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4
Q

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

A

Astrocytic gliosis

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5
Q

TSEs have a long or short incubation time?

A

long- really long

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6
Q

can a human survive a TSEs

A

no- invariably fatal

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7
Q

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

A

species barrier

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8
Q

TSEs are caused by

A

prion- misfolded protein
no DNA/no RNA

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9
Q

three routes of transmission for prion disease

A

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

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10
Q

Kuru causes

A

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

foodborne disease from ritual cannibalism

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11
Q

Kuru was probably caused by

A

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

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12
Q

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

A

iatrogenically (medical procedure)

infected equipment, human growth hormone from infected person

classical creutzfeldt-jakob disease

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13
Q

scrapie effect what animals

A

sheep and goats

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14
Q

scrapie presents with what symptoms

A

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

sheep and goat

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15
Q

what countries are scrapie free

A

australia and new zealand

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16
Q

how is scrapie spread

A

oral or via wounds in contaiminated pasture or by contact

17
Q

scrapie does not cause

A

inflammatory reaction

can not use serology to detect

18
Q

prion causes spongiforms by

A

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

19
Q

prions are resistant to

A

resistant to heat, nucleases, Xrays and UV

20
Q

what is the structure of prion protein

A

normal is alpha helical

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

21
Q

prions are partially resistant to —

A

protease

22
Q

how to test for prion

A

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

23
Q

PMCA can detect — but can’t detect —

A

Protein Misfolding Cyclic Amplification

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

24
Q

what is the normal function of prion proteins?

A

no one really knows

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

25
Q

BSE causes —

A

nervousness, skin tremors, manic kicking during milking, loss of condition, death in a few weeks

mad cow disease
Bovine spongiform encephalopathy

25
Q

BSE causes —

A

nervousness, skin tremors, manic kicking during milking, loss of condition, death in a few weeks

mad cow disease
Bovine spongiform encephalopathy

26
Q

what caused BSE

A

contaminated feed (meat and bone meal)
spontaneous mutation

27
Q

what is meat and bone meal

A

animal waste the is rendered into a protein rich feed

28
Q

old or young people are more susceptibile to prion

A

young- age dependent

29
Q

three types of BSE prion

A

c-type: classical
Ltype and H type: atypical

30
Q

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

A

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

31
Q

average age for sporatic vs variableCJD

A

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

32
Q

lesion profiles are the same for —- but different for

A

vCJD and BSE
scrapies and sCJD

33
Q

transmissible mink encephalopathy was probably caused by

A

eating BSE infected cow

34
Q

chronic wasting disease infect —

A

deer and elk

35
Q

chronic wasting disease is spread by

A

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

36
Q

Asymptomatic deer excrete infectious prions in
feces, spreading what disease

A

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

Fall 22: micro (25 decks)

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