Prions - TSEs in animals

8 important questions on Prions - TSEs in animals

Properties of chronic wasting disease:

  • Disease in wild deer, primarily in western US
  • symptoms: drooling, difficulties with swallowing, weight loss

Where does prion stand for?

Proteinaceous infectious particle:
  • resistant to:
    • heat
    • radiation
    • proteolytic enzymes
    • nucleases
    • conventional desinfectants
  • two forms of prions -> normal or abnormal
    • PrP-c or PrP-sen -> normal
    • PrP-Sc or PrP-res -> abnormal

What are the abnormal prions, and what are their properties?

Abnormal = Prp-sc or Prp-resistant
  • 30% a-helix, 43% b-sheets
  • cytoplasmic
  • amyloid-like character
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What are the normal prions and what are their properties?

Normal -> PrP-c or PrP sensitive
  • cellular membrane glycoprotein
  • expressed on the surface of neuronal cells
  • 42% a-helix, 3% b-sheest
  • function unknown

What is the difference between encephalitis and prion diseases?

Viral encephalitis have inflammation response, prion diseases do not have inflammation responses

Name 4 detection strategies of TSE:

  1. MRI
  2. Classical histology: amyloid plaques and spongeform (-> loss of neurons and vacuolization)
  3. Immunodetection: western blot detection using anti-prion antibodies
  4. Prion PCR: PrP-Sc converts PrP-c into aggregated form -> ultrasound breaks down aggregates -> short chains again convert PrP-c into PrP-Sc

What are the physiological functions of PrP?

It is anti-apoptotic -> prevents cell death
anti oxidant
involved in transmembrane signal transduction

What happens in Alzheimer's disease, with regard to prions?

Alzheimer's disease is characterized by aggregated or beta-amyloid (Ab) protein -> forms amyloid plaques in the spaces between neurons.
No seeding activity observed -> not infectious

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