Review Article
Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging
Table 1
Genetic modulation of proteasome in different models and its effect on aging and disease.
| Subunit deficiency/overexpression | Function | Phenotype | Model | Reference |
| Rpt2 inactivation | ATPase
| Ubiquitin and -synuclein positive Lewy like intraneural inclusion in neurons and neurodegeneration | Mus musculus | [59] |
| Rpt3 inactivation | ATPase | TDP43, FUS accumulation, basophilic inclusion bodies in neurons, locomotor impairment, loss of neurons | Mus musculus | [60] |
| 5t deletion | Chymotrypsin-like proteolytic activity | Shortening of lifespan, accumulation of polyubiquitinated and oxidized proteins, aggravated age-related metabolic disorder | Mus musculus | [66] |
| Rpn11 overexpression | Deubiquitination of the proteasome substrate | Extension of lifespan, suppression of polyQ induced toxicity | Drosophila melanogaster | [15] |
| Rpn6 overexpression | Stabilizing the interaction between CP and RP | Extension of lifespan under mild stress condition | Caenorhabditis elegans | [16] |
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