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Filamin C dimerisation is regulated by HSPB7

Abstract:
The biomechanical properties and responses of tissues underpin a variety important of physiological functions and pathologies. In striated muscle, the actin-binding protein filamin C (FLNC) is a key protein whose variants causative for a wide range of cardiomyopathies and musculoskeletal pathologies. FLNC is a multi-functional protein that interacts with a variety of partners, however, how it is regulated at the molecular level is not well understood. Here we investigate its interaction with HSPB7, a cardiac-specific molecular chaperone whose absence is embryonically lethal. We find that FLNC and HSPB7 interact in cardiac tissue under biomechanical stress, forming a strong hetero-dimer whose structure we solve by X-ray crystallography. Our quantitative analyses show that the hetero-dimer out-competes the FLNC homo-dimer interface, potentially acting to abrogate the ability of the protein to cross-link the actin cytoskeleton, and to enhance its diffusive mobility. We show that phosphorylation of FLNC at threonine 2677, located at the dimer interface and associated with cardiac stress, acts to favour the homo-dimer. Conversely, phosphorylation at tyrosine 2683, also at the dimer interface, has the opposite effect and shifts the equilibrium towards the hetero-dimer. Evolutionary analysis and ancestral sequence reconstruction reveals this interaction and its mechanisms of regulation to date around the time primitive hearts evolved in chordates. Our work therefore shows, structurally, how HSPB7 acts as a specific molecular chaperone that regulates FLNC dimerisation.
Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1038/s41467-025-58889-x

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Institution:
University of Oxford
Division:
MPLS
Department:
Chemistry
Role:
Author
ORCID:
0000-0002-6416-996X
More by this author
Institution:
University of Oxford
Division:
MPLS
Department:
Chemistry
Role:
Author
More by this author
Institution:
University of Oxford
Division:
MPLS
Department:
Chemistry
Role:
Author
More by this author
Institution:
University of Oxford
Division:
MPLS
Department:
Chemistry
Role:
Author


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Funder identifier:
https://ror.org/029chgv08
Grant:
201543/B/16/Z
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Funder identifier:
https://ror.org/012mzw131
Grant:
RPG-2021-246
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Funder identifier:
https://ror.org/02wdwnk04
Grant:
FS/12/40/29712
AA/18/2/34218
IA/F/23/275037
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Funder identifier:
https://ror.org/03x94j517
Grant:
MR/V009540/1
More from this funder
Funder identifier:
https://ror.org/03wnrjx87


Publisher:
Springer Nature
Journal:
Nature Communications More from this journal
Volume:
16
Issue:
1
Article number:
4090
Publication date:
2025-05-01
Acceptance date:
2025-04-02
DOI:
EISSN:
2041-1723
ISSN:
2041-1723


Language:
English
Pubs id:
2118135
Local pid:
pubs:2118135
Deposit date:
2025-04-14
ARK identifier:

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