Table 1.
Examples of mammalian and human disease-associated misfolded proteins that have been studied in yeast.
| Misfolded protein(s) |
Localization | Disease | Type |
|---|---|---|---|
| Huntingtin (Htt) | Cytoplasmic or nuclear | Huntington’s disease (HD) | Heritable |
| α-Synuclein (αSyn) | Cytoplasmic | Parkinson’s disease (PD) and other synucleinopathies | Mostly sporadic, sometimes heritable |
| Amyloid β (Aβ) | Mostly extracellular | Alzheimer’s disease (AD) | Primarily sporadic, rarely heritable |
| Tau | Cytoplasmic | AD, frontotemporal dementia (FTD), Pick’s disease and other tauopathies | Sporadic or heritable |
| Prion protein (PrP) | Mostly extracellular | Transmissible spongiform encephalopathies (TSEs) | Sporadic, heritable and/or infectious |
| TDP-43 | Cytoplasmic or nuclear | Amyotrophic lateral sclerosis (ALS), FTD | Sporadic or heritable |
| FUS | Cytoplasmic or nuclear | ALS | Sporadic or heritable |
| C9orf72 | Cytoplasmic | ALS, FTD | Heritable |
| Cu,Zn-superoxide dismutase (Sod1) | Cytoplasmic, or mitochondrial IMSa | ALS | Sporadic or heritable |
| hnRNPA2B1, hnRNPA1 | Nuclear or cytoplasmic | Multisystem proteinopathy (MPS)—type of ALS | Heritable |
| CREST | Nuclear | ALS | Heritable |
| VAPB, VCP, OPTN, SETX, profilin 1 | Cytoplasmic or nuclear | ALS | Heritable |
| Ataxin 2 (ATXN2) | Nuclear or cytoplasmic | Spinocereberral ataxia 2 (SCA), ALS, FTD | Heritable |
| Transthyretin (TTR) | Extracellular or cytoplasmic | Transthyretin amyloidosis (ATTR), polyneuropathy | Mostly heritable |
| Amylin (IAPP) | Mostly extracellular | Type II diabetes | Sporadic |
aIntramembrane space.