Gene therapy targeting protein trafficking regulator MOG1 in mouse models of Brugada syndrome, arrhythmias, and mild cardiomyopathy
G Yu, S Chakrabarti, M Tischenko, AL Chen… - Science Translational …, 2022 - science.org
Brugada syndrome (BrS) is a fatal arrhythmia that causes an estimated 4% of all sudden
death in high-incidence areas. SCN5A encodes cardiac sodium channel NaV1. 5 and …
death in high-incidence areas. SCN5A encodes cardiac sodium channel NaV1. 5 and …
Reduced Na+ and higher K+ channel expression and function contribute to right ventricular origin of arrhythmias in Scn5a+/− mice
CA Martin, U Siedlecka, K Kemmerich… - Open …, 2012 - royalsocietypublishing.org
Brugada syndrome (BrS) is associated with ventricular tachycardia originating particularly in
the right ventricle (RV). We explore electrophysiological features predisposing to such …
the right ventricle (RV). We explore electrophysiological features predisposing to such …
[HTML][HTML] 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 …
syndrome (BrS), an arrhythmic disorder that is a leading cause of sudden death and lacks …
MOG1 Rescues Defective Trafficking of Nav1.5 Mutations in Brugada Syndrome and Sick Sinus Syndrome
S Chakrabarti, X Wu, Z Yang, L Wu… - Circulation …, 2013 - Am Heart Assoc
Background—Loss-of-function mutations in Nav1. 5 cause sodium channelopathies,
including Brugada syndrome, dilated cardiomyopathy, and sick sinus syndrome; however …
including Brugada syndrome, dilated cardiomyopathy, and sick sinus syndrome; however …
[HTML][HTML] A common genetic variant within SCN10A modulates cardiac SCN5A expression
M Van Den Boogaard, S Smemo… - The Journal of …, 2014 - Am Soc Clin Investig
Variants in SCN10A, which encodes a voltage-gated sodium channel, are associated with
alterations of cardiac conduction parameters and the cardiac rhythm disorder Brugada …
alterations of cardiac conduction parameters and the cardiac rhythm disorder Brugada …
Functional epicardial conduction disturbances due to a SCN5A variant associated with Brugada syndrome
Background Brugada syndrome is a significant cause of sudden cardiac death (SCD), but
the underlying mechanisms remain hypothetical. Objectives This study aimed to elucidate …
the underlying mechanisms remain hypothetical. Objectives This study aimed to elucidate …
MOG1 A New Susceptibility Gene for Brugada Syndrome
D Kattygnarath, S Maugenre, N Neyroud… - Circulation …, 2011 - Am Heart Assoc
Background—Brugada syndrome (BrS) is caused mainly by mutations in the SCN5A gene,
which encodes the α-subunit of the cardiac sodium channel Nav1. 5. However,≈ 20% of …
which encodes the α-subunit of the cardiac sodium channel Nav1. 5. However,≈ 20% of …
[HTML][HTML] Genetic and molecular mechanisms in brugada syndrome
Brugada syndrome is a rare hereditary arrhythmia disorder characterized by a distinctive
electrocardiogram pattern and an elevated risk of ventricular arrhythmias and sudden …
electrocardiogram pattern and an elevated risk of ventricular arrhythmias and sudden …
[HTML][HTML] Role of SCN5A coding and non-coding sequences in Brugada syndrome onset: What's behind the scenes?
H Daimi, AH Khelil, A Neji, KB Hamda, S Maaoui… - biomedical …, 2019 - Elsevier
Abstract Background Brugada syndrome (BrS) is a rare inherited cardiac arrhythmia
associated with a high risk of sudden cardiac death (SCD) due to ventricular fibrillation (VF) …
associated with a high risk of sudden cardiac death (SCD) due to ventricular fibrillation (VF) …
[HTML][HTML] Embryonic type Na+ channel β-subunit, SCN3B masks the disease phenotype of Brugada syndrome
SCN5A is abundant in heart and has a major role in I Na. Loss-of-function mutation in
SCN5A results in Brugada syndrome (BrS), which causes sudden death in adults. It remains …
SCN5A results in Brugada syndrome (BrS), which causes sudden death in adults. It remains …
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