Understanding arrhythmogenic cardiomyopathy: advances through the use of human pluripotent stem cell models

CJ Chua, J Morrissette-McAlmon, L Tung, KR Boheler - Genes, 2023 - mdpi.com
Cardiomyopathies (CMPs) represent a significant healthcare burden and are a major cause
of heart failure leading to premature death. Several CMPs are now recognized to have a …

Basic and translational mechanisms in inflammatory arrhythmogenic cardiomyopathy

M Engel, EA Shiel, SP Chelko - International Journal of Cardiology, 2024 - Elsevier
Arrhythmogenic cardiomyopathy (ACM) is a familial, nonischemic heart disease typically
inherited via an autosomal dominant pattern (Nava et al.,[1]; Wlodarska et al.,[2]). Often …

NFĸB signaling drives myocardial injury via CCR2+ macrophages in a preclinical model of arrhythmogenic cardiomyopathy

SP Chelko, VR Penna, M Engel… - The Journal of …, 2024 - Am Soc Clin Investig
Nuclear factor κ-B (NFκB) is activated in iPSC-cardiac myocytes from patients with
arrhythmogenic cardiomyopathy (ACM) under basal conditions, and inhibition of NFκB …

PDMS–PEG block copolymer and pretreatment for arresting drug absorption in microphysiological devices

DB Mair, MAC Williams, JF Chen… - … Applied Materials & …, 2022 - ACS Publications
Poly (dimethylsiloxane)(PDMS) is a commonly used polymer in organ-on-a-chip devices
and microphysiological systems. However, due to its hydrophobicity and permeability, it …

Fibroblast growth factor homologous factors serve as a molecular rheostat in tuning arrhythmogenic cardiac late sodium current

N Chakouri, S Rivas, D Roybal, L Yang… - Nature cardiovascular …, 2022 - nature.com
Voltage-gated sodium channels (Nav channels) support the genesis and brisk spatial
propagation of action potentials in the heart. Disruption of NaV1. 5 inactivation results in a …

[HTML][HTML] Multifactorial approaches to enhance maturation of human iPSC-derived cardiomyocytes

K Kistamás, A Müller, S Muenthaisong… - Journal of Molecular …, 2023 - Elsevier
Cardiovascular diseases are the leading cause of death worldwide. Several strategies–
small molecules, gene therapy, surgeries, cardiac rehabilitation–have been developed to …

Arrhythmogenic Cardiomyopathy: from Preclinical Models to Genotype–phenotype Correlation and Pathophysiology

X Fan, G Yang, F Duru, M Grilli, I Akin, X Zhou… - Stem Cell Reviews and …, 2023 - Springer
Arrhythmogenic cardiomyopathy (ACM) is a hereditary myocardial disease characterized by
the replacement of the ventricular myocardium with fibrous fatty deposits. ACM is usually …

[HTML][HTML] Engineered tissue geometry and Plakophilin-2 regulate electrophysiology of human iPSC-derived cardiomyocytes

DW Simmons, G Malayath, DR Schuftan, J Guo… - APL …, 2024 - pubs.aip.org
Engineered heart tissues have been created to study cardiac biology and disease in a
setting that more closely mimics in vivo heart muscle than 2D monolayer culture. Previously …

Autophagy and endoplasmic reticulum stress during onset and progression of arrhythmogenic cardiomyopathy

M Pitsch, S Kant, C Mytzka, RE Leube, CA Krusche - Cells, 2021 - mdpi.com
Arrhythmogenic cardiomyopathy (AC) is a heritable, potentially lethal disease without a
causal therapy. AC is characterized by focal cardiomyocyte death followed by inflammation …

Mechanisms of innate immune injury in arrhythmogenic cardiomyopathy

SP Chelko, V Penna, M Engel, M Landim-Vieira… - bioRxiv, 2023 - biorxiv.org
Inhibition of nuclear factor kappa-B (NF kappa B) signaling prevents disease in
Dsg2mut/mut mice, a model of arrhythmogenic cardiomyopathy (ACM). Moreover, NF kappa …