Cardiac sodium channel complexes and arrhythmia: structural and functional roles of the ß1 and ß3 subunits.
J Physiol
; 601(5): 923-940, 2023 03.
Article
em En
| MEDLINE
| ID: mdl-36354758
ABSTRACT
In cardiac myocytes, the voltage-gated sodium channel NaV 1.5 opens in response to membrane depolarisation and initiates the action potential. The NaV 1.5 channel is typically associated with regulatory ß-subunits that modify gating and trafficking behaviour. These ß-subunits contain a single extracellular immunoglobulin (Ig) domain, a single transmembrane α-helix and an intracellular region. Here we focus on the role of the ß1 and ß3 subunits in regulating NaV 1.5. We catalogue ß1 and ß3 domain specific mutations that have been associated with inherited cardiac arrhythmia, including Brugada syndrome, long QT syndrome, atrial fibrillation and sudden death. We discuss how new structural insights into these proteins raises new questions about physiological function.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Arritmias Cardíacas
/
Síndrome do QT Longo
Limite:
Humans
Idioma:
En
Revista:
J Physiol
Ano de publicação:
2023
Tipo de documento:
Article