The prion model for progression and diversity of neurodegenerative diseases

Barbara E. Stopschinski, Marc I. Diamond

Research output: Contribution to journalReview article

54 Citations (Scopus)

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

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Prions
Neurodegenerative Diseases
Protein Conformation
Prion Diseases
Huntington Disease
Brain
Immunotherapy
Parkinson Disease
Alzheimer Disease
Pathology
Proteins
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

The prion model for progression and diversity of neurodegenerative diseases. / Stopschinski, Barbara E.; Diamond, Marc I.

In: The Lancet Neurology, Vol. 16, No. 4, 01.04.2017, p. 323-332.

Research output: Contribution to journalReview article

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