Lamin A/C Cardiomyopathy: Implications for Treatment.
Arrhythmias
CRISPR–Cas9 therapy
Heart failure
Lamin A/C gene
Laminopathy
Mechanotransduction
P53
Journal
Current cardiology reports
ISSN: 1534-3170
Titre abrégé: Curr Cardiol Rep
Pays: United States
ID NLM: 100888969
Informations de publication
Date de publication:
26 11 2019
26 11 2019
Historique:
entrez:
28
11
2019
pubmed:
28
11
2019
medline:
28
5
2020
Statut:
epublish
Résumé
The purpose of this review is to provide an update on lamin A/C (LMNA)-related cardiomyopathy and discuss the current recommendations and progress in the management of this disease. LMNA-related cardiomyopathy, an inherited autosomal dominant disease, is one of the most common causes of dilated cardiomyopathy and is characterized by steady progression toward heart failure and high risks of arrhythmias and sudden cardiac death. We discuss recent advances in the understanding of the molecular mechanisms of the disease including altered cell biomechanics, which may represent novel therapeutic targets to advance the current management approach, which relies on standard heart failure recommendations. Future therapeutic approaches include repurposed molecularly directed drugs, siRNA-based gene silencing, and genome editing. LMNA-related cardiomyopathy is the focus of active in vitro and in vivo research, which is expected to generate novel biomarkers and identify new therapeutic targets. LMNA-related cardiomyopathy trials are currently underway.
Identifiants
pubmed: 31773301
doi: 10.1007/s11886-019-1224-7
pii: 10.1007/s11886-019-1224-7
doi:
Substances chimiques
Lamin Type A
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
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