Abstract
RNA granules are microscopically visible cellular structures that aggregate by protein–protein and protein–RNA interactions. Using stress granules as an example, we discuss the principles of RNA granule formation, which rely on the multivalency of RNA and multi-domain proteins as well as low-affinity interactions between proteins with prion-like/low-complexity domains (e.g. FUS and TDP-43). We then explore how dysregulation of RNA granule formation is linked to neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), and discuss possible strategies for therapeutic intervention.
| Original language | English (US) |
|---|---|
| Title of host publication | Advances in Experimental Medicine and Biology |
| Publisher | Springer New York LLC |
| Pages | 263-296 |
| Number of pages | 34 |
| Volume | 907 |
| DOIs | |
| State | Published - 2016 |
Publication series
| Name | Advances in Experimental Medicine and Biology |
|---|---|
| Volume | 907 |
| ISSN (Print) | 00652598 |
| ISSN (Electronic) | 22148019 |
Keywords
- Low-complexity region
- Phase separation
- Prion-like domain
- RNA granules
- Stress granules
ASJC Scopus subject areas
- Medicine(all)
- Biochemistry, Genetics and Molecular Biology(all)
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Cite this
Fan, A. C., & Leung, A. K. L. (2016). RNA granules and diseases: A case study of stress granules in ALS and FTLD. In Advances in Experimental Medicine and Biology (Vol. 907, pp. 263-296). (Advances in Experimental Medicine and Biology; Vol. 907). Springer New York LLC. https://doi.org/10.1007/978-3-319-29073-7_11