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 …

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 …

Update on genetic basis of Brugada syndrome: monogenic, polygenic or oligogenic?

O Campuzano, G Sarquella-Brugada, S Cesar… - International Journal of …, 2020 - mdpi.com
Brugada syndrome is a rare inherited arrhythmogenic disease leading to ventricular
fibrillation and high risk of sudden death. In 1998, this syndrome was linked with a genetic …

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 …

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 …

[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 …

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 …

Genetic and molecular mechanisms in brugada syndrome

E Moras, K Gandhi, B Narasimhan, R Brugada… - Cells, 2023 - mdpi.com
Brugada syndrome is a rare hereditary arrhythmia disorder characterized by a distinctive
electrocardiogram pattern and an elevated risk of ventricular arrhythmias and sudden …

Brugada syndrome: oligogenic or mendelian disease?

MM Monasky, E Micaglio, G Ciconte… - International Journal of …, 2020 - mdpi.com
Brugada syndrome (BrS) is diagnosed by a coved-type ST-segment elevation in the right
precordial leads on the electrocardiogram (ECG), and it is associated with an increased risk …

Loss of sodium current caused by a Brugada syndrome–associated variant is determined by patient-specific genetic background

R Martínez-Moreno, D Carreras, G Sarquella-Brugada… - Heart Rhythm, 2024 - Elsevier
Abstract Background Brugada syndrome (BrS) is an inherited cardiac arrhythmogenic
disease that predisposes patients to sudden cardiac death. It is associated with mutations in …