Associated Papers
Genomic stop codon scanning reveals quantitative principles of nonsense-mediated mRNA decay.
Cortázar MA et al.
Biorxiv : the Preprint Server for Biology, 2025
Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy.
Dzianisava V et al.
Molecular Therapy. Nucleic Acids, 2025
Perinuclear organelle trauma at the nexus of cardiomyopathy pathogenesis arising from loss of function LMNA mutation.
Choi JC
Nucleus (austin, Tex.), 2025
LMNA-related cardiomyopathy: From molecular pathology to cardiac gene therapy.
Wang Z et al.
Journal of Advanced Research, 2025
Microtubule forces drive nuclear damage in LMNA cardiomyopathy.
Amiad Pavlov D et al.
Nature Cardiovascular Research, 2025
Precise gene editing of pathogenic Lamin A mutations corrects cardiac disease.
Caravia XM et al.
Proceedings of the National Academy of Sciences of the United States of America, 2025
The dual guardians of cellular stability: exploring nesprin and lamin in senescence.
Zhou ZY et al.
Cell Death & Disease, 2025
Nuclear envelope rupture in cardiomyocytes orchestrates early transcriptomic changes and immune activation in LMNA -DCM that are reversed by LINC complex disruption.
Zuela-Sopilniak N et al.
Biorxiv : the Preprint Server for Biology, 2025
Drosophila as a Model for Studying the Roles of Lamins in Normal Tissues and Laminopathies.
Zielińska A et al.
Cells, 2025
Dilated cardiomyopathy: from genes and molecules to potential treatments.
Wang X et al.
Molecular and Cellular Biochemistry, 2025
Cardiac fibroblasts counterbalance cardiomyocytes in LMNA cardiomyopathy pathogenesis.
Sikder K et al.
Biorxiv : the Preprint Server for Biology, 2025
Microtubule forces drive nuclear damage in LMNA cardiomyopathy.
Pavlov DA et al.
Biorxiv : the Preprint Server for Biology, 2025
A genetic variant in SMAD7 acts as a modifier of LMNA-associated muscular dystrophy, implicating SMAD signaling as a therapeutic target.
Mohar NP et al.
Science Advances, 2025
Domain-specific association of single-nucleotide variants in the LMNA gene with the phenotypic expression of dilated cardiomyopathy.
Zhuo W et al.
International Journal of Cardiology, 2025
G protein regulation by RGS proteins in the pathophysiology of dilated cardiomyopathy.
Garcia YE et al.
American Journal of Physiology. Heart and Circulatory Physiology, 2025
Role of Nuclear Lamins in the Regulation of the Genome: Focus on CardioLaminopathy.
Kervella M et al.
Sub-cellular Biochemistry, 2025
Changes in Chromatin Organisation and Mechanotransduction Mediated by Mutant Lamins in Laminopathies.
Nath S et al.
Sub-cellular Biochemistry, 2025
Cardiomyopathy: pathogenesis and therapeutic interventions.
Huang S et al.
Medcomm, 2024
Recent insights in striated muscle laminopathies.
Leconte M et al.
Current Opinion in Neurology, 2024
LMNA-Related Dilated Cardiomyopathy: Single-Cell Transcriptomics during Patient-Derived iPSC Differentiation Support Cell Type and Lineage-Specific Dysregulation of Gene Expression and Development for Cardiomyocytes and Epicardium-Derived Cells with Lamin A/C Haploinsufficiency.
Zaragoza MV et al.
Cells, 2024
Genetic and Pathophysiological Basis of Cardiac and Skeletal Muscle Laminopathies.
Bhide S et al.
Genes, 2024
The Pathogenic Mechanisms of and Novel Therapies for Lamin A/C-Related Dilated Cardiomyopathy Based on Patient-Specific Pluripotent Stem Cell Platforms and Animal Models.
Wu XY et al.
Pharmaceuticals (basel, Switzerland), 2024
Characteristics of nuclear architectural abnormalities of myotubes differentiated from LmnaH222P/H222P skeletal muscle cells.
Wada E et al.
In Vitro Cellular & Developmental Biology. Animal, 2024
Correction to: Ryanodine receptor remodeling in cardiomyopathy and muscular dystrophy caused by lamin A/C gene mutation.
Unknown Author
Human Molecular Genetics, 2024
LMNA -Related Dilated Cardiomyopathy: Single-Cell Transcriptomics during Patient-derived iPSC Differentiation Support Cell type and Lineage-specific Dysregulation of Gene Expression and Development for Cardiomyocytes and Epicardium-Derived Cells with Lamin A/C Haploinsufficiency.
Zaragoza MV et al.
Biorxiv : the Preprint Server for Biology, 2024
Global Proteomic Analysis Reveals Alterations in Differentially Expressed Proteins between Cardiopathic Lamin A/C Mutations.
Anderson CL et al.
Journal of Proteome Research, 2024
Eliminating elevated p53 signaling fails to rescue skeletal muscle defects or extend survival in lamin A/C-deficient mice.
Kirby TJ et al.
Cell Death Discovery, 2024
Perinuclear damage from nuclear envelope deterioration elicits stress responses that contribute to LMNA cardiomyopathy.
Sikder K et al.
Science Advances, 2024
Proteomic characterization of human LMNA-related congenital muscular dystrophy muscle cells.
Storey EC et al.
Neuromuscular Disorders : Nmd, 2024
mTOR Inhibition Prolongs Survival and Has Beneficial Effects on Heart Function After Onset of Lamin A/C Gene Mutation Cardiomyopathy in Mice.
Wu W et al.
Circulation. Heart Failure, 2024
From gene to mechanics: a comprehensive insight into the mechanobiology of LMNA mutations in cardiomyopathy.
Veltrop RJA et al.
Cell Communication and Signaling : Ccs, 2024
The structure and function of lamin A/C: Special focus on cardiomyopathy and therapeutic interventions.
Tiwari V et al.
Life Sciences, 2024
Primate Model Carrying LMNA Mutation Develops Dilated Cardiomyopathy.
Luo X et al.
Jacc. Basic to Translational Science, 2024
LMNA-related muscular dystrophy involving myoblast proliferation and apoptosis through the FOXO1/GADD45A pathway.
Wu Y et al.
Biochimica Et Biophysica Acta. Molecular Basis of Disease, 2024
Desmin and Plectin Recruitment to the Nucleus and Nuclei Orientation Are Lost in Emery-Dreifuss Muscular Dystrophy Myoblasts Subjected to Mechanical Stimulation.
Cenni V et al.
Cells, 2024
Intermediate filaments in the heart: The dynamic duo of desmin and lamins orchestrates mechanical force transmission.
West G et al.
Current Opinion in Cell Biology, 2023
Emerin deficiency does not exacerbate cardiomyopathy in a murine model of Emery-Dreifuss muscular dystrophy caused by an LMNA gene mutation.
Wada E et al.
The Journal of Physiological Sciences : Jps, 2023
Scaffold, mechanics and functions of nuclear lamins.
Buxboim A et al.
Febs Letters, 2023
Pharmacotherapeutic Approaches to Treatment of Muscular Dystrophies.
Rawls A et al.
Biomolecules, 2023
Systematic in vivo candidate evaluation uncovers therapeutic targets for LMNA dilated cardiomyopathy and risk of Lamin A toxicity.
Tan CY et al.
Journal of Translational Medicine, 2023
An alpha-helix variant p.Arg156Pro in LMNA as a cause of hereditary dilated cardiomyopathy: genetics and bioinfomatics exploration.
Chang L et al.
Bmc Medical Genomics, 2023
New drug discovery of cardiac anti-arrhythmic drugs: insights in animal models.
Sharma AK et al.
Scientific Reports, 2023
Nuclear proteostasis imbalance in laminopathy-associated premature aging diseases.
Pande S et al.
Faseb Journal : Official Publication of the Federation of American Societies for Experimental Biology, 2023
Caenorhabditis elegans models for striated muscle disorders caused by missense variants of human LMNA.
Gregory EF et al.
Plos Genetics, 2023
Lamin A/C Ablation Restricted to Vascular Smooth Muscle Cells, Cardiomyocytes, and Cardiac Fibroblasts Causes Cardiac and Vascular Dysfunction.
Del Monte-Monge A et al.
International Journal of Molecular Sciences, 2023
Net39 protects muscle nuclei from mechanical stress during the pathogenesis of Emery-Dreifuss muscular dystrophy.
Zhang Y et al.
The Journal of Clinical Investigation, 2023
Nesprin-1: novel regulator of striated muscle nuclear positioning and mechanotransduction.
De Silva S et al.
Biochemical Society Transactions, 2023
Perinuclear damage from nuclear envelope deterioration elicits stress responses that contribute to LMNA cardiomyopathy.
Sikder K et al.
Biorxiv : the Preprint Server for Biology, 2023