Disease Associated CaM Variants Interact with KCNQ1 to Confer Aberrant Membrane Trafficking and Function
Biophysical journal(2022)
摘要
Calmodulin (CaM) is a ubiquitous auxiliary subunit and a potent modulator of key ion channels underlying the cardiac action potential (AP). Missense variants in calmodulin (CaM) have emerged to underly arrhythmias associated with high mortality rates. As CaM regulates several key cardiac ion channels, mechanistic understanding of CaM variant-associated arrhythmias requires elucidating individual CaM variant's effect on distinct channels. One key CaM regulatory target is the KCNQ1 (KV7.1) voltage-gated potassium channel that underly the repolarizing IKs current.
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