Key result
Golgi trafficking-defective Nav1.5 mutants associated with Brugada syndrome concomitantly trap Kir2.1/2.2 channels, producing a dominant negative effect that unexpectedly decreases IK1 in addition to INa.
Why the study?
Does the presence of Brugada syndrome-associated Nav1.5 mutations alter IK1 density concomitantly with INa density in cardiac models?
Population
Mouse models of SCN5A haploinsufficiency, CHO cells, rat ventricular cardiomyocytes, and human induced…
Comparison
Expression of Brugada syndrome-associated… vs Wild-type Nav1.5 channels or…
Design
Preclinical
Authors
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May extend Brugada mechanisms to dual INa/IK1 loss; leaves open clinical relevance.
Does the presence of Brugada syndrome-associated Nav1.5 mutations alter IK1 density concomitantly with INa density in cardiac models?
Brugada syndrome-associated Nav1.5 mutations with Golgi trafficking defects can trap Kir2.1/2.2 channels, leading to a concomitant decrease in both INa and IK1 currents, which may exacerbate arrhythmogenesis.
Pérez-Hernández et al. (2018) studied Brugada syndrome. Nav1.5 trafficking-defective mutations vs. Wild-type Nav1.5 channels was evaluated on IK1 and INa current densities. Golgi trafficking-defective Nav1.5 mutants associated with Brugada syndrome concomitantly trap Kir2.1/2.2 channels, producing a dominant negative effect that unexpectedly decreases IK1 in addition to INa.
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