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RBM20, a potential target for treatment of cardiomyopathy via titin isoform switching

Wei Guo1,2, Mingming Sun3,4

  • 1Animal Science, University of Wyoming, Laramie, WY, 82071, USA. wguo3@uwyo.edu.

Biophysical Reviews
|June 4, 2017
PubMed

Insights

Titin protein

Area of Science:

  • Cardiovascular Biology
  • Molecular Cardiology
  • Sarcomere Protein Dynamics

Background:

  • Cardiomyopathy, or heart muscle disease, involves impaired heart function.
  • Titin, a giant sarcomeric protein, influences heart muscle stiffness and is linked to cardiomyopathy.
  • Mechanisms of titin's role in heart disease, including genetic mutations and splicing, require further elucidation.

Purpose of the Study:

  • To review the role of titin (TTN) isoform switching in cardiomyopathy.
  • To explore the regulation of TTN splicing by RBM20.
  • To discuss TTN splicing's impact on heart diastolic stiffness and systolic compliance.

Main Methods:

  • Literature review focusing on titin biology and cardiomyopathy.
  • Analysis of TTN alternative splicing mechanisms.
  • Discussion of RBM20's regulatory role in TTN gene expression.

Main Results:

  • Titin isoform switching and TTN mutations affect passive myocardial stiffness.
  • RBM20 is a key regulator of TTN splicing.
  • Altered TTN splicing impacts cardiac diastolic and systolic function in health and disease.

Conclusions:

  • Titin's role in cardiomyopathy is multifaceted, involving isoform variation and genetic mutations.
  • RBM20-mediated TTN splicing is crucial for regulating cardiac mechanics.
  • Targeting RBM20 offers potential therapeutic strategies for cardiomyopathy and heart failure.
Keywords:
Alternative splicingCardiomyopathyRBM20Titin isoform switching

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