Prion-Like Proteins in Phase Separation and Their Link to Disease
Aberrant protein folding underpins many neurodegenerative diseases as well as certain myopathies and cancers. Protein misfolding can be driven by the presence of distinctive prion and prion-like regions within certain proteins. These prion and prion-like regions have also been found to drive liquid-...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-07-01
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| Series: | Biomolecules |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2218-273X/11/7/1014 |
| _version_ | 1797527508579516416 |
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| author | Macy L. Sprunger Meredith E. Jackrel |
| author_facet | Macy L. Sprunger Meredith E. Jackrel |
| author_sort | Macy L. Sprunger |
| collection | DOAJ |
| description | Aberrant protein folding underpins many neurodegenerative diseases as well as certain myopathies and cancers. Protein misfolding can be driven by the presence of distinctive prion and prion-like regions within certain proteins. These prion and prion-like regions have also been found to drive liquid-liquid phase separation. Liquid-liquid phase separation is thought to be an important physiological process, but one that is prone to malfunction. Thus, aberrant liquid-to-solid phase transitions may drive protein aggregation and fibrillization, which could give rise to pathological inclusions. Here, we review prions and prion-like proteins, their roles in phase separation and disease, as well as potential therapeutic approaches to counter aberrant phase transitions. |
| first_indexed | 2024-03-10T09:44:42Z |
| format | Article |
| id | doaj.art-1a4b7d9ff13c436284603bd02e9aa88b |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-10T09:44:42Z |
| publishDate | 2021-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-1a4b7d9ff13c436284603bd02e9aa88b2023-11-22T03:18:48ZengMDPI AGBiomolecules2218-273X2021-07-01117101410.3390/biom11071014Prion-Like Proteins in Phase Separation and Their Link to DiseaseMacy L. Sprunger0Meredith E. Jackrel1Department of Chemistry, Washington University, St. Louis, MO 63130, USADepartment of Chemistry, Washington University, St. Louis, MO 63130, USAAberrant protein folding underpins many neurodegenerative diseases as well as certain myopathies and cancers. Protein misfolding can be driven by the presence of distinctive prion and prion-like regions within certain proteins. These prion and prion-like regions have also been found to drive liquid-liquid phase separation. Liquid-liquid phase separation is thought to be an important physiological process, but one that is prone to malfunction. Thus, aberrant liquid-to-solid phase transitions may drive protein aggregation and fibrillization, which could give rise to pathological inclusions. Here, we review prions and prion-like proteins, their roles in phase separation and disease, as well as potential therapeutic approaches to counter aberrant phase transitions.https://www.mdpi.com/2218-273X/11/7/1014prionsprion-like domainsamyloidprotein misfoldingliquid-liquid phase separationaberrant phase transitions |
| spellingShingle | Macy L. Sprunger Meredith E. Jackrel Prion-Like Proteins in Phase Separation and Their Link to Disease Biomolecules prions prion-like domains amyloid protein misfolding liquid-liquid phase separation aberrant phase transitions |
| title | Prion-Like Proteins in Phase Separation and Their Link to Disease |
| title_full | Prion-Like Proteins in Phase Separation and Their Link to Disease |
| title_fullStr | Prion-Like Proteins in Phase Separation and Their Link to Disease |
| title_full_unstemmed | Prion-Like Proteins in Phase Separation and Their Link to Disease |
| title_short | Prion-Like Proteins in Phase Separation and Their Link to Disease |
| title_sort | prion like proteins in phase separation and their link to disease |
| topic | prions prion-like domains amyloid protein misfolding liquid-liquid phase separation aberrant phase transitions |
| url | https://www.mdpi.com/2218-273X/11/7/1014 |
| work_keys_str_mv | AT macylsprunger prionlikeproteinsinphaseseparationandtheirlinktodisease AT meredithejackrel prionlikeproteinsinphaseseparationandtheirlinktodisease |