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

Alexander C. Fan, Anthony K.L. Leung

Research output: Chapter in Book/Report/Conference proceedingChapter

27 Scopus citations


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
Number of pages34
StatePublished - 2016

Publication series

NameAdvances in Experimental Medicine and Biology
ISSN (Print)0065-2598
ISSN (Electronic)2214-8019


  • Low-complexity region
  • Phase separation
  • Prion-like domain
  • RNA granules
  • Stress granules

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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