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micobiology/immuno Section 3 > scary prions > Flashcards

scary prions Flashcards

1
Q

what PrPsc types have been seen in the classical forms of CJD?

A

1-3

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2
Q

how do prions “replicate”

A

once PrPsc is formed, the nucleus (“seed”) recruits other PrPc and converts them to PrPsc.
from there, nucleus increases in size to become an amyloid fiber
fragmentation then occurs

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3
Q

how do you test for CJD?

A

brain biopsy is golden standard

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4
Q

what types of medical tests will be abnormal in a person with CJD?

A

MRI

EKG

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5
Q

what is the brain biopsy of a person with sCJD characterized by?

A

accumulation of PrPsc
neuronal loss without inflammation
spongiform change

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6
Q

in sCJD, what is the average disease duration? when does death usually occur?

A

disease duration is 5-6 months

death usually occurs within 1 year of symptom onset

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7
Q

what age group does sCJD seem to present in?

A

older population

median age is 68

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8
Q

what prion type causes vCJD?

A

type 4

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9
Q

compare the disease progression of vCJD, sCJD and Alzheimer’s

A

alzherimer disease progression is much slower than the two types of CJD. sCJD has the FASTEST disease progression

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10
Q

what is the illness duration of vCJD?

A

14 months

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11
Q

what tissues have prominent involvement in vCJD that is not seen in sCJD (or iCJD)?

A

prominent involvement of lymphoreticular tissues

vCDJ has special tropism for lymphoid tissues

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12
Q

list some differences between sCJD and vCJD that can allow for differentiation?

A

type of PrPsc
MRI signal is different from one another
in patients with sCJD there is abnormal PrP deposition
in cCJD florid PrP plaques consist of a round amyloid core surrounded by spongiform VACUOLES!

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13
Q

mean age of onset for vCJD?

A

29 years

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14
Q

how is vCJD transmitted?

A

person-person through blood transfusion or ingestion of food contaminated with BSE

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15
Q

what is the average incubation period for vCJD?

A

20-30 years!

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16
Q

list new targets for CJD treatment

A
  • PrP13: breaks the beta sheet confirmation and has been shown to delay symptoms
  • anti-PrP monoclonal antibodies
  • adenovirus vector platforms that express PrPc single chain fragment
17
Q

what two animals is the disease scarpie seen in?

A

sheep and goat

18
Q

what two animals is chronic wasting disease disease?

A

deer and elk

19
Q

list the 5 prion diseases seen in humans

A 
kuru
fatal familial insomnia (FFI)
GSS syndrome
vCJD
sCJD
20
Q

what is the cause of sCJD and what percentage of CJD does it make up?

A

no known cause 85-95%

21
Q

what is the cause of fCJD and what percentage of CJD does it make up?

A

inherited genetic risk 7-10%

22
Q

what is the cause of iCJD and what percentage of CJD does it make up?

A

exposure during medical procedures <1%

23
Q

what are the 2 cardinal clinical manifestations of sCJD?

A

metal deterioration and myoclonus

24
Q

what gene encodes for the prion protein?

A

Prnp gene

25
Q

what is the structure of PrPc?

A

alpha helix

26
Q

what is the structure of PrPsc?

A

beta sheet

27
Q

what are the proposed functions of PrPc?

A

maintain white matter, regulate innate immune cells, respond to oxidative stress and neuron formation

28
Q

how were the 4 types of CJD found?

A

by observing different fragment sizes observed in western blots following treatment with proteinase K

29
Q

Can sCJD be transported between person to person (blood transfusions)? What about eating BSE contaminated meat?

A

NO

30
Q

What is myoclonus? Who is it seen in ?

A

It is an un-voluntary muscle jerk

Seen in patients with sCJD

31
Q

List 3 things that have led to the protein only hypothesis

A

No nucleus Acids
Agent is resisted to UV radiation and nuclear es
No immune response to infection

32
Q

List 3 reasons why Alzheimer’s is thought to be linked to prions

A
  1. Polymorphism in prp gene is a risk Factor for AD
  2. PrPc binds to amyloid B peptides
  3. Mice injected with brain tissue from AD patient will develop disease

micobiology/immuno Section 3 (12 decks)

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