Article ; Online: The structure and function of lamin A/C: Special focus on cardiomyopathy and therapeutic interventions.
2024 Volume 341, Page(s) 122489
Abstract: Lamins are inner nuclear membrane proteins that belong to the intermediate filament family. Lamin A/C lie adjacent to the heterochromatin structure in polymer form, providing skeletal to the nucleus. Based on the localization, lamin A/C provides nuclear ... ...
Abstract | Lamins are inner nuclear membrane proteins that belong to the intermediate filament family. Lamin A/C lie adjacent to the heterochromatin structure in polymer form, providing skeletal to the nucleus. Based on the localization, lamin A/C provides nuclear stability and cytoskeleton to the nucleus and modulates chromatin organization and gene expression. Besides being the structural protein making the inner nuclear membrane in polymer form, lamin A/C functions as a signalling molecule involved in gene expression as an enhancer inside the nucleus. Lamin A/C regulates various cellular pathways like autophagy and energy balance in the cytoplasm. Its expression is highly variable in differentiated tissues, higher in hard tissues like bone and muscle cells, and lower in soft tissues like the liver and brain. In muscle cells, including the heart, lamin A/C must be expressed in a balanced state. Lamin A/C mutation is linked with various diseases, such as muscular dystrophy, lipodystrophy, and cardiomyopathies. It has been observed that a good number of mutations in the LMNA gene impact cardiac activity and its function. Although several works have been published, there are still several unexplored areas left regarding the lamin A/C function and structure in the cardiovascular system and its pathological state. In this review, we focus on the structural organization, expression pattern, and function of lamin A/C, its interacting partners, and the pathophysiology associated with mutations in the lamin A/C gene, with special emphasis on cardiovascular diseases. With the recent finding on lamin A/C, we have summarized the possible therapeutic interventions to treat cardiovascular symptoms and reverse the molecular changes. |
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MeSH term(s) | Humans ; Lamin Type A/genetics ; Lamin Type A/chemistry ; Lamin Type A/metabolism ; Cardiomyopathies/genetics ; Cardiomyopathies/therapy ; Muscular Dystrophies/genetics ; Muscular Dystrophies/pathology ; Mutation ; Polymers |
Chemical Substances | Lamin Type A ; Polymers |
Language | English |
Publishing date | 2024-02-08 |
Publishing country | Netherlands |
Document type | Journal Article ; Review |
ZDB-ID | 3378-9 |
ISSN | 1879-0631 ; 0024-3205 |
ISSN (online) | 1879-0631 |
ISSN | 0024-3205 |
DOI | 10.1016/j.lfs.2024.122489 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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