Prions Flashcards

1
Q

t/f are prions viruses?

A

FALSE

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2
Q

types of prions

A
  • transmissible spongiform encephalopathies
  • bovine spongiform encephalopathy
  • scrapie
  • Chronic wasting disease
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3
Q

mad cow disease

A

bovine spongiform encephalopathy
transmitted by feeding cow meat and bone meal to other cows
strict rules govern animal feed and screening of animals is done

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4
Q

what has scrapie

A

sheep and goats

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5
Q

chronic eating disease

A

deer, elk, antelope

transmitted through cuts in the skin

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6
Q

how does human disease from prions come

A

from eating nervous tissue or the tongue of diseased animals

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7
Q

Creutzfeldt-Jakob disease

A
in humans
rare
degenerative
invariably fatal brain disorder
about 1 per million persons
can be sporadic, hereditary or acquired
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8
Q

sporadic Creutzfeldt- jakob disease

A

at least 85% of the cases

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9
Q

acquired Creutzfeldt- jakob disease

A

from transplants

<1% of cases

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10
Q

inherited Creutzfeldt- jakob disease

A

from a mutant proteins

5-10% of cases in the US

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11
Q

vCJD outbreak

A

in the UK from beef in the 90s and killed 177

eating meat, tongue of diseased animals

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12
Q

Kuru

A

cannibalism
from eating human brains
papua New Guinea

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13
Q

diagnosis

A

patients referred due to behavioral changes
must rule out other causes of dementia including treatable encephalitis and meningitis
by protein detection from biopsies or brain sectioning on autopsy

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14
Q

diseases by prions

A

progressive disease; have vacuolization of the grey matter of the brain
long term- 20 or more years until symptoms
neurological impairment- anxiety, memory loss, mode changes etc
movement problems: muscle twitching, spasms
loss of speech, coma and death

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15
Q

can prions be detected by Antibodies

A

no

because they do not induce an immune response

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16
Q

prion disease treatment

A

NONE

17
Q

Prions are

A

Single proteins found in the nervous system on the surface on the neurons in the brain

18
Q

What happens when prions contact normal cellular proteins

A

They contact and induce a normal cellular protein to change shape and to become a prion protien

19
Q

Infectious protein hypothesis

A

When a normal protein converts to the prion form

PrPc= the prion cellular counterpart
PrPres= prions
The prions turn only their own cellular counterpart into prions
They will not, do not affect other proteins

20
Q

Affected regions of the human brain that are affected by different TSEs

A
  • Cerebrum
  • Thalamus
  • spinal cord
  • cerebellum
21
Q

Proteins prions proteins are extremely hard to inactivate. Why?

A

Resistant to:

  1. Routine autoclaving
  2. Proteases
  3. Organic solvents
  4. Alkaline cleaners
  5. UV light
  6. Radiation
  7. Ethanol
  8. Formaldehyde

To inactivate need:
1N NaOH or undiluted bleach
And autoclaving at 121C for 1 hour

22
Q

What is TSE

A

Transmissible spongiform encephalopathy