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Epigenetics in LMNA-Related Cardiomyopathy

Mutations in the gene for lamin A/C (LMNA) cause a diverse range of diseases known as laminopathies. LMNA-related cardiomyopathy is a common inherited heart disease and is highly penetrant with a poor prognosis. In the past years, numerous investigations using mouse models, stem cell technologies, a...

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Autores principales: Wang, Yinuo, Dobreva, Gergana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10001118/
https://www.ncbi.nlm.nih.gov/pubmed/36899919
http://dx.doi.org/10.3390/cells12050783
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author Wang, Yinuo
Dobreva, Gergana
author_facet Wang, Yinuo
Dobreva, Gergana
author_sort Wang, Yinuo
collection PubMed
description Mutations in the gene for lamin A/C (LMNA) cause a diverse range of diseases known as laminopathies. LMNA-related cardiomyopathy is a common inherited heart disease and is highly penetrant with a poor prognosis. In the past years, numerous investigations using mouse models, stem cell technologies, and patient samples have characterized the phenotypic diversity caused by specific LMNA variants and contributed to understanding the molecular mechanisms underlying the pathogenesis of heart disease. As a component of the nuclear envelope, LMNA regulates nuclear mechanostability and function, chromatin organization, and gene transcription. This review will focus on the different cardiomyopathies caused by LMNA mutations, address the role of LMNA in chromatin organization and gene regulation, and discuss how these processes go awry in heart disease.
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spelling pubmed-100011182023-03-11 Epigenetics in LMNA-Related Cardiomyopathy Wang, Yinuo Dobreva, Gergana Cells Review Mutations in the gene for lamin A/C (LMNA) cause a diverse range of diseases known as laminopathies. LMNA-related cardiomyopathy is a common inherited heart disease and is highly penetrant with a poor prognosis. In the past years, numerous investigations using mouse models, stem cell technologies, and patient samples have characterized the phenotypic diversity caused by specific LMNA variants and contributed to understanding the molecular mechanisms underlying the pathogenesis of heart disease. As a component of the nuclear envelope, LMNA regulates nuclear mechanostability and function, chromatin organization, and gene transcription. This review will focus on the different cardiomyopathies caused by LMNA mutations, address the role of LMNA in chromatin organization and gene regulation, and discuss how these processes go awry in heart disease. MDPI 2023-03-01 /pmc/articles/PMC10001118/ /pubmed/36899919 http://dx.doi.org/10.3390/cells12050783 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Wang, Yinuo
Dobreva, Gergana
Epigenetics in LMNA-Related Cardiomyopathy
title Epigenetics in LMNA-Related Cardiomyopathy
title_full Epigenetics in LMNA-Related Cardiomyopathy
title_fullStr Epigenetics in LMNA-Related Cardiomyopathy
title_full_unstemmed Epigenetics in LMNA-Related Cardiomyopathy
title_short Epigenetics in LMNA-Related Cardiomyopathy
title_sort epigenetics in lmna-related cardiomyopathy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10001118/
https://www.ncbi.nlm.nih.gov/pubmed/36899919
http://dx.doi.org/10.3390/cells12050783
work_keys_str_mv AT wangyinuo epigeneticsinlmnarelatedcardiomyopathy
AT dobrevagergana epigeneticsinlmnarelatedcardiomyopathy