About the book
Despite intensive research, prion diseases still remain fatal neurodegenerative disorders. Since the discovery of the host-encoded cellular prion protein PrPC over 30 years ago, much advance has been made to define PrPC as a critical mediator of prion-induced neuronal death and to delineate some mechanisms of neurodegeneration occurring in prion diseases and in other amyloid diseases like Alzheimer’s. PrPC has been identified as a receptor for Aβ oligomeric species (Aβo) that also relays Aβo toxicity. The Aβo/PrPC interaction notably provokes synaptic damage in Alzheimer’s brain, supporting the view of an Aβo pathocascade that connects Aβ and tau pathologies. In line with this, soluble tau seeds would transmit amyloid-β pathology and potentially Alzheimer’s disease and behave as prionoids that propagate and spread from cell to cell.
The emergence of novel prion strains in animals, which include the only evidenced zoonotic prion C-BSE causing vCJD in humans, has created an important public health concern. Currently, new threats to human and animals may develop because of the plausible zoonotic properties of scrapie, L-BSE and the recently emerging chronic wasting disease in Europe.
This book will gather experts in prion diseases and present new scientific advances in the field and relations with other amyloid neuropathologies.