Twists and turns in gating ion channels with voltage

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Science  12 Aug 2016:
Vol. 353, Issue 6300, pp. 646-647
DOI: 10.1126/science.aah4194

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The discovery of mutations that make flies shake or dance was a major breakthrough in neuroscience (1) because it led to the identification of genes for an array of ion channels that are selectively permeable to potassium ions. The gene mutated in the dancing flies, christened “Ether-a-go-go” (EAG) because the flies move like go-go dancers in response to ether (2), encodes the founding member of the KCNH family of voltage-activated potassium (Kv) channels, which play critical roles in regulating excitability, irregular heartbeat (cardiac long QT syndrome), epilepsy, and cancer (3). On page 664 of this issue, Whicher and MacKinnon (4) solve the structure of the EAG (KCNH1) Kv channel bound to calmodulin (CaM) and discover an unanticipated mechanism by which voltage controls the process of channel opening and closing.