Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Apr;16(4):323-332.
doi: 10.1016/S1474-4422(17)30037-6. Epub 2017 Feb 24.

The Prion Model for Progression and Diversity of Neurodegenerative Diseases

Affiliations
Review

The Prion Model for Progression and Diversity of Neurodegenerative Diseases

Barbara E Stopschinski et al. Lancet Neurol. .

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.

Comment in

Similar articles

See all similar articles

Cited by 30 articles

See all "Cited by" articles

LinkOut - more resources

Feedback