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virology > Prions > Flashcards

Prions Flashcards

1
Q

nucleic acid or proteins?

A

Proteins

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2
Q

what does prion stand for?

A

protein of infectious nature, proteinaceous infectious particle

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3
Q

Morphology

A
  • pure protein (253 Aa in human prion)

- the infectious particle does not contain nucleic acid

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4
Q

resistance

A

extremely high
(138°C for 20 min) concentrated NaOH or formaldehyde
-lipid solvents reduce heat resistance

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5
Q

Biological properties

A
  • Cellular prion protein (PrP^c)

- Infectious prion protein (PrP^sc)

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6
Q

Cellular prion protein (PrP^c)

A
  • healthy, produced in the body - encoded in the chromosomes
  • normal part of the neurons (cytoskeleton, messenger)
  • coded on the 20th chromosome in humans
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7
Q

Infectious prion protein (PrP^sc)

A
  • sc = scrapie - bad prion -> infection in animals and humans
  • PrP^c and PrP^sc differ in 3 aa in human
  • isoform: alpha-helix -> beta-sheet
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8
Q

Problem with infectious prion proteins

A

resistant to protease -> accumulation in neurons (not part of the normal balance: productiondigestion)

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9
Q

! Pathogenesis !

A

intestine -> spleen, lymph nodes (6mnd) -> nerves

reach the CNS (after a long time) - modulator proteins: induce neurons to produce PrP^sc

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10
Q

What does the pathogenesis lead to?

A

intracellular accumulation, aggregation, polymerization

-amyloid plaques (3-4 years) - neuron degeneration -> spongiform encephalopathy

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11
Q

Clinical signs

A

progressive CNS signs, behaviour changes (aggression, anxiety, etc. eventually death) -> slowly developing, non-curable lethal disease

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12
Q

Antigenic properties

A

not antigentic - recognized as own -> no antibodies produced -> no immune responce

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13
Q

Diagnosis

A

clinical signs + histopathology: vacuolization of neurons, amyloid plaques, spongiform (brain looks like a sponge)

1st: ELISA test
2nd: Western blot, or proteinase K digestion + IF

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14
Q

TSEs =

A

transmissible spongiform encephalopathies

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15
Q

Scrapie

A

sheep: incoordination, swaying, itching

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16
Q

BSE = bovine spongiform encephalopathy

A

scrapie modification? (cows fed sheep protein to induce milk production)
-> behavior changes, “mad cow syndome”

17
Q 
FSE = feline spongiform encephalopathy 
TMe = transmissible mink encephalopathy
A

BSE modification?

both lead to decreased hygene, defecate everywere etc.

18
Q

CWD = chronic wasting disease

A

mule, deer, elk (North America): starve, die because of CNS problems

19
Q

How does prions affect humans

A

progressive dementia

20
Q

Creutzfeld-Jakob disease (CJD)

A

familial, sporadic (inherited), iatrogenic

21
Q

variant Creutzfeld-Jakob disease (vCJD)

A

BSE origin? (earlier only people over 40-50 years old were affected, now all the way down to 20s)

22
Q

GSS =?

A

Gerstmann-Srtäussler-Scheinker

23
Q

Kuru

A

laughing death - due to ritual cannibalism

virology (12 decks)

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