Telomere shortening in laminopathic dilated cardiomyopathy

Laminopathies are a group of rare disease due to mutations in the LMNA gene, which is crucial for nuclear integrity and cellular rigidity. Depending on the mutation, the disease manifests in striated muscles, adipose tissues, nerves, and the heart. Although many laminopathic patients exhibit accelerated aging syndromes, the connection as to why loss of LMNA drives aging remains unknown. Herein, we present evidence that cardiomyocytes from laminopathic heart sections exhibit shortened telomeres. Patient derived hiPSC-CMs we observed LMNA mutation results in myocardial enlargement and altered contractility in cardiomyocytes. Further, laminopathic murine cardiomyocytes recapitulates telomere attrition phenotype.

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Publication Details

Journal
npj Regenerative Medicine
Published
2026-02-10
DOI
https://doi.org/10.1038/s41536-026-00462-1
Primary Topic
Nuclear Structure and Function
Type
article
Field-Weighted Citation Impact
0.00

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article

Telomere shortening in laminopathic dilated cardiomyopathy

Anna Kirillova, Luka Nicin, Bonne Gisèle, Foster Birnbaum et al.
npj Regenerative Medicine
Nuclear Structure and Function
article

Telomere shortening in laminopathic dilated cardiomyopathy

Anna Kirillova, Luka Nicin, Bonne Gisèle, Foster Birnbaum, Helen M. Blau, Gaspard Pardon, Vittavat Termglinchan, Andrew C. H. Chang, Annie Laquerrière, Alex C. Y. Chang, Joseph Wu, Chenguang Wang
article en

Abstract

Laminopathies are a group of rare disease due to mutations in the LMNA gene, which is crucial for nuclear integrity and cellular rigidity. Depending on the mutation, the disease manifests in striated muscles, adipose tissues, nerves, and the heart. Although many laminopathic patients exhibit accelerated aging syndromes, the connection as to why loss of LMNA drives aging remains unknown. Herein, we present evidence that cardiomyocytes from laminopathic heart sections exhibit shortened telomeres. Patient derived hiPSC-CMs we observed LMNA mutation results in myocardial enlargement and altered contractility in cardiomyocytes. Further, laminopathic murine cardiomyocytes recapitulates telomere attrition phenotype.

npj Regenerative Medicine
Inserm (FR), Shanghai Jiao Tong University (CN), Cardiovascular Institute of the South (US), Sorbonne Université (FR), Institute for Stem Cell Biology and Regenerative Medicine (IN), Shanghai Ninth People's Hospital (CN), Institut de Myologie (FR), Stanford Medicine (US), Centre de Recherche en Myologie, Stanford University (US), Baxter (United States) (US)
National Science Foundation, American Heart Association, Li Ka Shing Foundation, Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung, National Natural Science Foundation of China, National Institutes of Health, Program for Professor of Special Appointment (Eastern Scholar) at Shanghai Institutions of Higher Learning
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