4.7 Article

Modulation of cytoskeleton in cardiomyopathy caused by mutations in LMNA gene

期刊

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
卷 324, 期 6, 页码 C1223-C1235

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AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajpcell.00471.2022

关键词

A -type lamins; cytoskeleton; intermediate fi laments; LMNA cardiomyopathy; nuclear envelope

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Dilated cardiomyopathy caused by LMNA mutations is a common cause of sudden death and heart failure. A-type lamins play a crucial role in maintaining nuclear structure and organizing the cytoskeleton. LMNA mutations can disrupt various structural components and cytoskeletal proteins, leading to poor cardiac contractility. Targeting cytoskeletal dynamics could be a potential therapeutic approach for LMNA cardiomyopathy.
Dilated cardiomyopathy caused by mutations in LMNA, encoding A-type lamins (i.e., LMNA cardiomyopathy), is characterized by a left ventricle enlargement and ultimately results in poor cardiac contractility associated with conduction defects. Despite current strategies to aggressively manage the symptoms, the disorder remains a common cause of sudden death and heart failure with decreased ejection fraction. Patient care includes cardioverter defibrillator implantation but the last therapeutic option remains cardiac transplantation. A-type lamins are intermediate filaments and are the main components of the nuclear lamina, a meshwork underlying the inner nuclear membrane, which plays an essential role in both maintaining the nuclear structure and organizing the cytoskeletal structures within the cell. Cytoskeletal proteins function as scaffold to resist external mechanical stress. An increasing amount of evidence demonstrates that LMNA mutations can lead to disturbances in several structural and cytoskeletal components of the cell such as microtubules, actin cytoskeleton, and intermediate filaments. Collectively, this review focuses on the significance of these cytoskeletal modulators and emphasizes their potential therapeutic role in LMNA cardiomyopathy. Indeed, molecular tuning of cytoskeletal dynamics has been successfully used in preclinical models and provides adequate grounds for a therapeutic approach for patients with LMNA cardiomyopathy.

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