Abstract

Human ether-á-go-go-related gene (hERG) potassium channels play a key role in the regulation of cardiac repolarization, neuronal excitability, and tumorigenesis. hERG channels contain N-terminal Per-Arnt-Sim (PAS) and C-terminal cyclic nucleotide-binding homology (CNBH) domains. Structural, functional and fluorescence-based studies established that the hERG PAS and CNBH domains from adjacent subunits interact. Moreover, many long-QT syndrome (LQTS)-linked mutations are located at the interface between these domains.

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