Table 1.
Neurodegenerative diseases that are associated with protein misfolding
Neurodegenerative diseases |
Aggregation- prone protein |
Neuronal population lost |
Disease symptoms |
Current therapies |
Refs |
---|---|---|---|---|---|
Polyglutamine disorders: Huntington’s disease, spinal and bulbar muscular atrophy, spinocerebellar ataxias and others |
Polyglutamine tracts within distinct pathogenic proteins |
Variable | Chorea, cognitive decline, depression and anxiety, dementia and ataxia |
Tetrabenazine, amantadine, remacemide, antipsychotics and antidepressants |
13 |
Parkinson’s disease | α-synuclein | Substantia nigra pars compacta, ventral tegmental area, autonomic ganglia and neurons of the myenteric plexus |
Tremors, bradykinesia, postural instability, rigidity, dementia, depression, anxiety, gastrointestinal dysfunction and hallucinations |
Levodopa/ carbidopa, dopamine agonists and monoamine oxidase inhibitors |
7,8 |
Alzheimer’s disease | Amyloid-β, hyperphosphory- lated tau |
Cortical and subcortical neurons, locus coeruleus and cholinergic neurons |
Learning and memory impairments, motor dysfunction, and irritability |
Acetylcholin esterase inhibitors, memantine and antipsychotics |
9,10 |
Amyotrophic lateral sclerosis/ Lou Gehrig’s disease |
Superoxide dismutase, TAR DNA binding protein 43 and others |
Motor neurons of the cortex, brain stem and spinal cord |
Muscular atrophy and motor dysfunction |
Riluzole | 11 |
Prion disorders: Creutzfeldt–Jakob disease, Gerstmann– Straussler syndrome, fatal familial insomnia and kuru |
Various forms of the prion protein such as scrapie (PrPSc) |
Variable | Spongiform encephalopathy, personality changes, depression and insomnia |
None | 12 |