RNA granules and diseases: A case study of stress granules in ALS and FTLD

Alexander C. Fan, Anthony Kar Lun Leung

Research output: Chapter in Book/Report/Conference proceedingChapter

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 languageEnglish (US)
Title of host publicationAdvances in Experimental Medicine and Biology
PublisherSpringer New York LLC
Pages263-296
Number of pages34
Volume907
DOIs
StatePublished - 2016

Publication series

NameAdvances in Experimental Medicine and Biology
Volume907
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