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Lamin A/C Cardiomyopathies: Current Understanding and Novel Treatment Strategies

  • Heart Failure (W Tang, Section Editor)
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Opinion statement

Dilated cardiomyopathy (DCM) is the third leading cause of heart failure in the USA. A major gene associated with DCM with cardiac conduction system disease is lamin A/C (LMNA) gene. Lamins are type V filaments that serve a variety of roles, including nuclear structure support, DNA repair, cell signaling pathway mediation, and chromatin organization. In 1999, LMNA was found responsible for Emery-Dreifuss muscular dystrophy (EDMD) and, since then, has been found in association with a wide spectrum of diseases termed laminopathies, including LMNA cardiomyopathy. Patients with LMNA mutations have a poor prognosis and a higher risk for sudden cardiac death, along with other cardiac effects like dysrhythmias, development of congestive heart failure, and potential need of a pacemaker or ICD. As of now, there is no specific treatment for laminopathies, including LMNA cardiomyopathy, because the mechanism of LMNA mutations in humans is still unclear. This review discusses LMNA mutations and how they relate to DCM, the necessity for further investigation to better understand LMNA mutations, and potential treatment options ranging from clinical and therapeutic to cellular and molecular techniques.

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Acknowledgments

This work is supported by the National Institutes of Health (R01HL103931) and the Collins Family Fund.

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Authors and Affiliations

  1. Department of Cellular and Molecular Medicine, Lerner Research Institute, Cleveland, OH, USA

    Xi Wang MD, PhD, Allyson Zabell BA & W. H. Wilson Tang MD

  2. Children’s Hospital of Pittsburgh, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Wonshill Koh MD, PhD

  3. Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA

    W. H. Wilson Tang MD

  4. Center for Clinical Genomics, Cleveland Clinic, Cleveland, OH, USA

    W. H. Wilson Tang MD

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  1. Xi Wang MD, PhD
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  2. Allyson Zabell BA
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Correspondence to W. H. Wilson Tang MD.

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Xi Wang, Allyson Zabell, and Wonshill Koh each declare no potential conflicts of interest.

W. H. Wilson Tang reports grants from the National Institutes of Health. Dr. Tang is a section editor for Current Treatment Options in Cardiovascular Medicine.

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This article is part of the Topical Collection on Heart Failure

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Wang, X., Zabell, A., Koh, W. et al. Lamin A/C Cardiomyopathies: Current Understanding and Novel Treatment Strategies. Curr Treat Options Cardio Med 19, 21 (2017). https://doi.org/10.1007/s11936-017-0520-z

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