Table 1.
Misfolding of specific proteins causes a spectrum of neurodegenerative disorders
DisorderProtein aggregateSeedingIntercellular transmissibility
Prion diseases (CJD, vCJD, Kuru, and FFI) PrPSc Yes Yes 
AD Aβ, Tau Yes Yes 
Tauopathies (tauopathy, progressive supranuclear palsy, Pick’s disease, and frontotemporal dementia) Tau Yes Yes 
HD mHtt Yes Yes 
Synucleinopathies (PD, DLB, MSA, and PAF) α-Synuclein Yes Yes 
ALS TDP-43 Yes (Polymenidou and Cleveland, 2012) Yes (Feiler et al., 2015) 
 FUS Yes (Polymenidou and Cleveland, 2012)  
 SOD Yes (Polymenidou and Cleveland, 2012) Yes (Pokrishevsky et al., 2016) 
DisorderProtein aggregateSeedingIntercellular transmissibility
Prion diseases (CJD, vCJD, Kuru, and FFI) PrPSc Yes Yes 
AD Aβ, Tau Yes Yes 
Tauopathies (tauopathy, progressive supranuclear palsy, Pick’s disease, and frontotemporal dementia) Tau Yes Yes 
HD mHtt Yes Yes 
Synucleinopathies (PD, DLB, MSA, and PAF) α-Synuclein Yes Yes 
ALS TDP-43 Yes (Polymenidou and Cleveland, 2012) Yes (Feiler et al., 2015) 
 FUS Yes (Polymenidou and Cleveland, 2012)  
 SOD Yes (Polymenidou and Cleveland, 2012) Yes (Pokrishevsky et al., 2016) 

Although amyotrophic lateral sclerosis is not discussed in this review, evidence suggests that proteins involved in amyotrophic lateral sclerosis are prion-like. Evidence for seeding and transmissibility of other proteins are described in detail in the text. CJD, Creutzfeldt–Jakob disease; DLB, dementia with Lewy body; FFI, fatal familial insomnia; MSA, multiple system atrophy; PAF, pure autonomic failure; SOD, superoxide dismutase; vCJD, variant Creutzfeldt–Jakob disease.

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