Reproduction: (6)

• Prions spread by transmitting a misfolded protein state
• When a prion enters a healthy organism, it causes the existing properly folded proteins to convert to the disease-associated prion form
• To do this, the prion acts as a template to misfold more proteins
• In turn, each newly formed prion goes on to convert more proteins itself
• This triggers a chain reaction that produces large amounts of the prion form

 Pathology:
Pic is (15)

• Diseases caused by prions are called transmissible spongiform encephalopathies (TSEs) which are always fatal (2)
• Characteristic of infection include: loss of motor control, dementia, paralysis, encephalitis, widespread neuronal loss (2)
• The prion altered protein structure is extremely stable and accumulates in infected tissues, causing tissue damage and cell death (8)
• The stable structure of prions makes them hard to eliminate with chemical and physical agents, making it hard to dispose of or contain them (8)
• The main part of the brain affected by the Kuru prion is the Cerebellum (6)

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