Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • Federica F. Morelli - , University of Modena and Reggio Emilia (Author)
  • Dineke S. Verbeek - , University of Groningen (Author)
  • Jessika Bertacchini - , University of Modena and Reggio Emilia (Author)
  • Jonathan Vinet - , University of Modena and Reggio Emilia (Author)
  • Laura Mediani - , University of Modena and Reggio Emilia (Author)
  • Sandra Marmiroli - , University of Modena and Reggio Emilia (Author)
  • Giovanna Cenacchi - , University of Bologna (Author)
  • Milena Nasi - , University of Modena and Reggio Emilia (Author)
  • Sara De Biasi - , University of Modena and Reggio Emilia (Author)
  • Jeanette F. Brunsting - , University of Groningen (Author)
  • Jan Lammerding - , Cornell University (Author)
  • Elena Pegoraro - , University of Padua (Author)
  • Corrado Angelini - , San Camillo Hospital (Author)
  • Rossella Tupler - , University of Modena and Reggio Emilia (Author)
  • Simon Alberti - , Max Planck Institute of Molecular Cell Biology and Genetics (Author)
  • Serena Carra - , University of Modena and Reggio Emilia (Author)

Abstract

Small heat shock proteins (HSPBs) contain intrinsically disordered regions (IDRs), but the functions of these IDRs are still unknown. Here, we report that, in mammalian cells, HSPB2 phase separates to form nuclear compartments with liquid-like properties. We show that phase separation requires the disordered C-terminal domain of HSPB2. We further demonstrate that, in differentiating myoblasts, nuclear HSPB2 compartments sequester lamin A. Increasing the nuclear concentration of HSPB2 causes the formation of aberrant nuclear compartments that mislocalize lamin A and chromatin, with detrimental consequences for nuclear function and integrity. Importantly, phase separation of HSPB2 is regulated by HSPB3, but this ability is lost in two identified HSPB3 mutants that are associated with myopathy. Our results suggest that HSPB2 phase separation is involved in reorganizing the nucleoplasm during myoblast differentiation. Furthermore, these findings support the idea that aberrant HSPB2 phase separation, due to HSPB3 loss-of-function mutations, contributes to myopathy.

Details

Original languageEnglish
Pages (from-to)2100-2115
Number of pages16
JournalCell reports
Volume20
Issue number9
Publication statusPublished - 29 Aug 2017
Peer-reviewedYes
Externally publishedYes

External IDs

PubMed 28854361
ORCID /0000-0003-4017-6505/work/142253861

Keywords

Keywords

  • congenital myopathy, lamin-A/C, nucleus, phase transition, small HSPs

Library keywords