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HCM-Associated MuRF1 Variants Compromise Ubiquitylation and Are Predicted to Alter Protein Structure.

Jitpisute Chunthorng-Orn1,2, Maya Noureddine3, Peter W J Dawson1

  • 1School of Sport, Exercise and Rehabilitation Sciences, University of Birmingham, Birmingham B15 2TT, UK.

International Journal of Molecular Sciences
|May 7, 2025
PubMed
Summary

Twenty-five variants of muscle RING finger protein-1 (MuRF1) impair its ubiquitylation activity, impacting cardiac muscle function. Structural modeling reveals how these TRIM63 gene variants affect MuRF1

Keywords:
MuRF1MuRF1 domainsTRIM63hypertrophic cardiomyopathypathogenic variantsstructural modellingubiquitylation

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Area of Science:

  • Molecular Biology
  • Cardiovascular Research
  • Genetics

Background:

  • Muscle RING finger protein-1 (MuRF1), encoded by TRIM63, is a critical E3 ubiquitin ligase regulating cardiac muscle size and function.
  • Numerous TRIM63 variants are associated with hypertrophic (HCM) and restrictive cardiomyopathies, but their functional and structural consequences are largely unknown.

Purpose of the Study:

  • To investigate the functional impact of 25 identified MuRF1 variants on its ubiquitylation activity.
  • To predict the structural consequences of these MuRF1 variants using computational approaches.

Main Methods:

  • Site-directed PCR mutagenesis was used to generate MuRF1 variants.
  • In vitro ubiquitylation assays were performed using purified MuRF1 variants and a titin fragment.
  • Computational structural modeling was employed to predict the impact of variants on MuRF1 structure.

Main Results:

  • All 25 MuRF1 variants reduced its ability to monoubiquitylate a titin fragment.
  • Seventeen variants significantly impaired or abolished MuRF1 auto-monoubiquitylation.
  • Structural modeling indicated that 10 variants disrupt critical interactions, potentially compromising protein integrity, while others showed varied structural impacts.

Conclusions:

  • The study highlights the functional impairment of numerous MuRF1 variants in ubiquitylation.
  • Structural predictions offer insights into the mechanisms underlying variant pathogenicity in cardiomyopathies.
  • Functional and structural analyses are crucial for understanding the role of TRIM63 variants in cardiac disease.