Structure:
-PrP(Sc) the disease-linked misfolded form
- All known mammalian prion diseases are caused by the so-called prion protein PrP (9)
-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
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Sporadic
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Familial
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