@inbook{91cf2488661c4904bee25208cc2aa8e7,
title = "RNA granules and diseases: A case study of stress granules in ALS and FTLD",
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.",
keywords = "Low-complexity region, Phase separation, Prion-like domain, RNA granules, Stress granules",
author = "Fan, {Alexander C.} and Leung, {Anthony K.L.}",
note = "Funding Information: We would like to thank Drs. Phillip Sharp, Nancy Kedersha, Ana?s Aulas, and Voula Mili for critical reading of the manuscript, Drs. Steve McKnight and Masato Kato on sharing their insights on the role of low-complexity domains in RNA granule formation. This work was partly supported by Johns Hopkins Catalyst Award and NIH R01-GM104135 to A.K.L.L.",
year = "2016",
doi = "10.1007/978-3-319-29073-7_11",
language = "English (US)",
series = "Advances in Experimental Medicine and Biology",
publisher = "Springer New York LLC",
pages = "263--296",
booktitle = "Advances in Experimental Medicine and Biology",
}