Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1

Selma Gulyurtlu, Monika S. Magon, Patrick Neville Guest, Panagiotis P. Papavasiliou, Kim Morrison, Alan Prescott, Judith E. Sleeman*

*Corresponding author for this work

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Abstract

RNA regulation in mammalian cells requires complex physical compartmentalisation using structures thought to be formed by liquid-liquid phase separation. Disruption of these structures is implicated in numerous degenerative diseases. Myotonic Dystrophy Type 1 (DM1) is a multi-systemic trinucleotide repeat disorder resulting from a CTG expansion in the DM1 protein kinase gene (DMPK). The cellular hall-mark of DM1 is the formation of nuclear foci containing expanded DMPK RNA (CUGexp). We report here the deregulation of stress granules (SGs) and processing bodies (P-bodies), two cytoplasmic structures key for mRNA regulation, in cell culture models of DM1. Alterations to the rates of formation and dispersal of SGs suggest an altered ability of cells to respond to stress associated with DM1, while changes to the structure and dynamics of SGs and P-bodies suggest that a widespread alteration to the biophysical properties of cellular structures may be a consequence of the presence of CUGexp RNA.
Original languageEnglish
Article numberdmm049294
JournalDisease Models & Mechanisms
Volume15
Issue number7
DOIs
Publication statusPublished - 2 Aug 2022

Keywords

  • LLPS
  • Myotonic Dystrophy Type-1
  • P-bodies
  • Stress granules
  • Trinucleotide repeats

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