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Distinguishing Characteristics

A prion is a small infectious particle constructed of an abnormally folded protein that causes progressive neurodegenerative conditions (12)

Responsible for transmissible spongiform encephalopathies TSEs (2)

pic is (13)

Structure:
  • All known mammalian prion diseases are caused by the so-called prion protein PrP (9)
          -PrP(C) is the endogenous properly folded form
          -PrP(Sc) the disease-linked misfolded form
  • The exact structure is not known: can be formed by combining PrP(C), polyadenylic acid and lipids in a protein misfolding cyclic amplification (PMCA) (11)
  • The abnormal fold is called the amyloid fold, in which the protein is aggregated into densely packed beta sheets (12)
  • The polypeptide aggregates form plaques which are thought to cause "entanglement" of neurofibrils and interferes with synapse function, leading to neuron death and dementia (12)
Classification:   Each can be transmitted experimentally (2)
Infectious
  • spread by consumption of infected material, latrogenic spread (organ transplant especially cornea), transfusion
  • eg. Kuru, Mad Cow, Scrapie
Sporadic
  • 1-2 million people are infected worldwide, shows up later in life
  • evidence shows that some sporadic TSE is a result of infection
Familial
  • due to autosomal dominant mutation of PrP
  • inherited: at least 10-15% of total human TSE cases
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