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CHAPTER 6

Prion Disease

Prion diseases (derived either from infection, germline mutations or most often occurring sporadically), both in humans and animals, are fatal neurodegenerative disorders characterized by progressive brain degeneration. It is widely accepted that they are caused by protein‐only infectious agents propagating disease by inducing protein conformational changes. The molecular mechanism of prion pathologies is not yet entirely understood but some aspects seem to be generally accepted, such as spongiform degeneration, non‐classical inflammation of the brain, progressive neuron loss, accumulation of protein aggregates and synaptic alterations.

Publication details

Print publication date
24 Jul 2013
Copyright year
2013
Print ISBN
978-1-84973-588-9
PDF eISBN
978-1-84973-589-6

From the book series:
Metallobiology