[HTML][HTML] Modeling of lamin A/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells

CW Siu, YK Lee, JCY Ho, WH Lai, YC Chan… - Aging (Albany …, 2012 - ncbi.nlm.nih.gov
AIMS We identified an autosomal dominant non-sense mutation (R225X) in exon 4 of the
lamin A/C (LMNA) gene in a Chinese family spanning 3 generations with familial dilated …

Modeling treatment response for lamin A/C related dilated cardiomyopathy in human induced pluripotent stem cells

YK Lee, YM Lau, ZJ Cai, WH Lai, LY Wong… - Journal of the …, 2017 - Am Heart Assoc
Background Precision medicine is an emerging approach to disease treatment and
prevention that takes into account individual variability in the environment, lifestyle, and …

Modeling of LMNA-Related Dilated Cardiomyopathy Using Human Induced Pluripotent Stem Cells

D Shah, L Virtanen, C Prajapati, M Kiamehr, J Gullmets… - Cells, 2019 - mdpi.com
Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure and heart
transplantation. A portion of familial DCM is due to mutations in the LMNA gene encoding …

Activation of PDGF pathway links LMNA mutation to dilated cardiomyopathy

J Lee, V Termglinchan, S Diecke, I Itzhaki, CK Lam… - Nature, 2019 - nature.com
Abstract Lamin A/C (LMNA) is one of the most frequently mutated genes associated with
dilated cardiomyopathy (DCM). DCM related to mutations in LMNA is a common inherited …

Phenotypic Variability in iPSC-Induced Cardiomyocytes and Cardiac Fibroblasts Carrying Diverse LMNA Mutations

J Yang, MA Argenziano, M Burgos Angulo… - Frontiers in …, 2021 - frontiersin.org
Mutations in the LMNA gene (encoding lamin A/C) are a significant cause of familial
arrhythmogenic cardiomyopathy. Although the penetrance is high, there is considerable …

Clinical trial in a dish using iPSCs shows lovastatin improves endothelial dysfunction and cellular cross-talk in LMNA cardiomyopathy

N Sayed, C Liu, M Ameen, F Himmati… - Science translational …, 2020 - science.org
Mutations in LMNA, the gene that encodes lamin A and C, causes LMNA-related dilated
cardiomyopathy (DCM) or cardiolaminopathy. LMNA is expressed in endothelial cells (ECs); …

DNA damage response/TP53 pathway is activated and contributes to the pathogenesis of dilated cardiomyopathy associated with LMNA (Lamin A/C) mutations

SN Chen, R Lombardi, J Karmouch, JY Tsai… - Circulation …, 2019 - Am Heart Assoc
Rationale: Mutations in the LMNA gene, encoding LMNA (lamin A/C), are responsible for
laminopathies. Dilated cardiomyopathy (DCM) is a major cause of mortality and morbidity in …

Pathogenic LMNA variants disrupt cardiac lamina-chromatin interactions and de-repress alternative fate genes

PP Shah, W Lv, JH Rhoades, A Poleshko, D Abbey… - Cell Stem Cell, 2021 - cell.com
Pathogenic mutations in LAMIN A/C (LMNA) cause abnormal nuclear structure and
laminopathies. These diseases have myriad tissue-specific phenotypes, including dilated …

Plasma microRNAs as biomarkers for Lamin A/C-related dilated cardiomyopathy

R Toro, S Blasco-Turrión, FJ Morales-Ponce… - Journal of Molecular …, 2018 - Springer
Abstract Lamin A/C gene (LMNA)-related familial dilated cardiomyopathy (fDCM) is an
aggressive heart disease that often leads to transplantation and sudden death. The aim of …

Lamin A/C-dependent chromatin architecture safeguards naïve pluripotency to prevent aberrant cardiovascular cell fate and function

Y Wang, A Elsherbiny, L Kessler, J Cordero… - Nature …, 2022 - nature.com
Tight control of cell fate choices is crucial for normal development. Here we show that lamin
A/C plays a key role in chromatin organization in embryonic stem cells (ESCs), which …