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What are prions?


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Prions:

  1. Prion illnesses, often known as transmissible spongiform encephalopathies (TSEs), are a group of uncommon neurodegenerative conditions that affect humans and animals.
  2. Long incubation times, spongiform changes linked to neuronal death, and the failure to induce an inflammatory response are all symptoms.
  3. TSEs are hypothesized to be caused by prion proteins.
  4. The name "prions" refers to aberrant, pathogenic agents that are transmissible and can cause improper folding of specific normal cellular proteins known as prion proteins, which are abundant in the brain.
  5. These normal prion proteins' functions are currently being investigated. Prion proteins fold incorrectly, resulting in brain injury and the disease's classic signs and symptoms. Prion illnesses usually progress rapidly and are always fatal.
  6. Example: In humans-Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, Kuru
  7. In animals-Bovine Spongiform Encephalopathy (BSE), Chronic Wasting Disease (CWD), Scrapie, Transmissible mink encephalopathy, Feline spongiform encephalopathy, Ungulate spongiform encephalopathy.

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