Table 1.
Disease | Misfolded protein | Studies revealing evidence for self-propagation | Neuronal loss | Fatal disease |
---|---|---|---|---|
Prion diseases | PrPSc | 19,20 | Yes | Yes |
AD | Aβ | 81–84 | Yes | Yes |
Tau | 85 | Yes | Yes | |
PD | α-syn | 88 | Yes | Yes |
Multiple system atrophy | α-syn | 78 | Yes | Yes |
ALS | SOD1 | 93 | Yes | Yes |
TDP-43 | 96 | Yes | Yes | |
HD | PolyQ repeat | 95 | Yes | Yes |
Notes: Evidence of self-propagation of the causative agent in a number of neurodegenerative diseases. The majority of evidence comes from studies where brain homogenates from sick donor mice were injected either intracerebrally or intraperitoneally into recipient mice. Donor mice were analyzed for disease symptoms, accumulation, and self-propagation of the misfolded protein and, in some cases, travel of the misfolded protein from the injection site to other areas of the brain.
Abbreviations: AD, Alzheimer’s disease; PD, Parkinson’s disease; ALS, amyotrophic lateral sclerosis; HD, Huntington’s disease; SOD1, superoxide dismutase 1; PolyQ, polyglutamine.