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 language | English (US) |
|---|---|
| Pages (from-to) | 323-332 |
| Number of pages | 10 |
| Journal | The Lancet Neurology |
| Volume | 16 |
| Issue number | 4 |
| DOIs | |
| State | Published - Apr 1 2017 |
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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 journal › Review article
}
TY - JOUR
T1 - The prion model for progression and diversity of neurodegenerative diseases
AU - Stopschinski, Barbara E.
AU - Diamond, Marc I.
PY - 2017/4/1
Y1 - 2017/4/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85013662802&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85013662802&partnerID=8YFLogxK
U2 - 10.1016/S1474-4422(17)30037-6
DO - 10.1016/S1474-4422(17)30037-6
M3 - Review article
C2 - 28238712
AN - SCOPUS:85013662802
VL - 16
SP - 323
EP - 332
JO - The Lancet Neurology
JF - The Lancet Neurology
SN - 1474-4422
IS - 4
ER -