The prion model for progression and diversity of neurodegenerative diseases

Barbara E. Stopschinski, Marc I. Diamond

Research output: Contribution to journalReview article

62 Scopus citations

Abstract

The neuropathology of different neurodegenerative diseases begins in different brain regions, and involves distinct brain networks. Evidence indicates that transcellular propagation of protein aggregation, which is the basis of prion disease, might underlie the progression of pathology in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and Huntington's disease. The prion model predicts specific patterns of neuronal vulnerability and network involvement on the basis of the conformation of pathological proteins. Indeed, evidence indicates that self-propagating aggregate conformers, or so-called strains, are associated with distinct neuropathological syndromes. The extension of this hypothesis to our understanding of common neurodegenerative disorders can suggest new therapeutic approaches, such as immunotherapy and small molecules, to block transcellular propagation, and new diagnostic tools to detect early evidence of disease.

Original languageEnglish (US)
Pages (from-to)323-332
Number of pages10
JournalThe Lancet Neurology
Volume16
Issue number4
DOIs
StatePublished - Apr 1 2017

ASJC Scopus subject areas

  • Clinical Neurology

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