Patient-specific iPSC-derived cardiomyocytes reveal variable phenotypic severity of Brugada syndrome

Y Sun, J Su, X Wang, J Wang, F Guo, H Qiu, H Fan… - …, 2023 - thelancet.com
Summary Background Brugada syndrome (BrS) is a cardiac channelopathy that can result in
sudden cardiac death (SCD). SCN5A is the most frequent gene linked to BrS, but the …

Studying Brugada syndrome with an SCN1B variants in human-induced pluripotent stem cell-derived cardiomyocytes

I El-Battrawy, J Müller, Z Zhao, L Cyganek… - Frontiers in Cell and …, 2019 - frontiersin.org
Background Among rare channelopathies BrS patients are at high risk of sudden cardiac
death (SCD). SCN5A mutations are found in a quarter of patients. Other rare gene mutations …

A cellular model of Brugada syndrome with SCN10A variants using human-induced pluripotent stem cell-derived cardiomyocytes

I El-Battrawy, S Albers, L Cyganek, Z Zhao, H Lan… - EP …, 2019 - academic.oup.com
Abstract Aims Brugada syndrome (BrS) is associated with a pronounced risk to develop
sudden cardiac death (SCD). Up to 21% of patients are related to mutations in SCN5A …

Patient-specific and genome-edited induced pluripotent stem cell–derived cardiomyocytes elucidate single-cell phenotype of Brugada syndrome

P Liang, K Sallam, H Wu, Y Li, I Itzhaki, P Garg… - Journal of the American …, 2016 - jacc.org
Abstract Background: Brugada syndrome (BrS), a disorder associated with characteristic
electrocardiogram precordial ST-segment elevation, predisposes afflicted patients to …

Disease phenotypes and mechanisms of iPSC-derived cardiomyocytes from Brugada syndrome patients with a loss-of-function SCN5A mutation

W Li, M Stauske, X Luo, S Wagner… - Frontiers in cell and …, 2020 - frontiersin.org
Brugada syndrome (BrS) is one of the major causes of sudden cardiac death in young
people, while the underlying mechanisms are not completely understood. Here, we …

Patient-specific iPSC-derived cardiomyocytes reveal aberrant activation of Wnt/β-catenin signaling in SCN5A-related Brugada syndrome

D Cai, X Wang, Y Sun, H Fan, J Zhou, Z Yang… - Stem Cell Research & …, 2023 - Springer
Background Mutations in the cardiac sodium channel gene SCN5A cause Brugada
syndrome (BrS), an arrhythmic disorder that is a leading cause of sudden death and lacks …

Brugada syndrome: different experimental models and the role of human cardiomyocytes from induced pluripotent stem cells

Y Li, S Lang, I Akin, X Zhou… - Journal of the American …, 2022 - Am Heart Assoc
Brugada syndrome (BrS) is an inherited and rare cardiac arrhythmogenic disease
associated with an increased risk of ventricular fibrillation and sudden cardiac death …

hiPSC-derived cardiomyocytes from Brugada Syndrome patients without identified mutations do not exhibit clear cellular electrophysiological abnormalities

CC Veerman, I Mengarelli, K Guan, M Stauske… - Scientific reports, 2016 - nature.com
Brugada syndrome (BrS) is a rare cardiac rhythm disorder associated with sudden cardiac
death. Mutations in the sodium channel gene SCN5A are found in~ 20% of cases while …

Experimental models of Brugada syndrome

F Sendfeld, E Selga, FS Scornik, GJ Pérez… - International Journal of …, 2019 - mdpi.com
Brugada syndrome is an inherited, rare cardiac arrhythmogenic disease, associated with
sudden cardiac death. It accounts for up to 20% of sudden deaths in patients without …

[HTML][HTML] Sodium channel current loss of function in induced pluripotent stem cell-derived cardiomyocytes from a Brugada syndrome patient

E Selga, F Sendfeld, R Martinez-Moreno… - Journal of molecular and …, 2018 - Elsevier
Brugada syndrome predisposes to sudden death due to disruption of normal cardiac ion
channel function, yet our understanding of the underlying cellular mechanisms is …