Noncoding RNAs and Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes in Cardiac Arrhythmic Brugada Syndrome

B Theisen, A Holtz, V Rajagopalan - Cells, 2023 - mdpi.com
Hundreds of thousands of people die each year as a result of sudden cardiac death, and
many are due to heart rhythm disorders. One of the major causes of these arrhythmic events …

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 …

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 …

iPSC-cardiomyocyte models of Brugada syndrome—achievements, challenges and future perspectives

A Nijak, J Saenen, AJ Labro, D Schepers… - International journal of …, 2021 - mdpi.com
Brugada syndrome (BrS) is an inherited cardiac arrhythmia that predisposes to ventricular
fibrillation and sudden cardiac death. It originates from oligogenic alterations that affect …

[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 …

Single-cell transcriptomics trajectory and molecular convergence of clinically relevant mutations in Brugada syndrome

R Tambi, R Abdel Hameid… - American Journal …, 2021 - journals.physiology.org
Brugada syndrome (BrS) is a rare, inherited arrhythmia with high risk of sudden cardiac
death. To evaluate the molecular convergence of clinically relevant mutations and to identify …

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 …

HiPSC-derived cardiomyocyte to model Brugada syndrome: both asymptomatic and symptomatic mutation carriers reveal increased arrhythmogenicity

K Penttinen, C Prajapati, D Shah, DK Rajan… - BMC Cardiovascular …, 2023 - Springer
Brugada syndrome is an inherited cardiac arrhythmia disorder that is mainly associated with
mutations of the cardiac voltage-gated sodium channel alpha subunit 5 (SCN5A) gene. The …

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 …

RRAD mutation causes electrical and cytoskeletal defects in cardiomyocytes derived from a familial case of Brugada syndrome

N Belbachir, V Portero, ZR Al Sayed… - European Heart …, 2019 - academic.oup.com
Abstract Aims The Brugada syndrome (BrS) is an inherited cardiac disorder predisposing to
ventricular arrhythmias. Despite considerable efforts, its genetic basis and cellular …