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Prion Diseases and Dementia

  • Piero Parchi
  • Pierluigi Gambetti
Part of the Contemporary Neuroscience book series (CNEURO)

Abstract

Prion diseases are a group of neurodegenerative conditions of humans and animals that include sporadic, inherited, and transmitted forms. They are best characterized by their distinctive pathogenic mechanism, which is shared by all three forms. The central event in the pathogenesis of these diseases is thought to be a change in protein conformation that results in the conversion of a normal protein, identified as cellular prion protein (PrPC), into an isoform that is partially resistant to proteases (PrPres) (1). The molecular events leading to this conformational change, however, remain largely unknown.

Keywords

Prion Protein Prion Disease Progressive Dementia Fatal Familial Insomnia Human Prion Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Springer Science+Business Media New York 1997

Authors and Affiliations

  • Piero Parchi
  • Pierluigi Gambetti

There are no affiliations available

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