Pharmacologically, KV7.1 can be selectively activated by the benzodiazepine L-364,373 (R-L3), which is a partial agonist and increases the amplitude of KV7.1 currents as well as slowing the rate of channel activation and deactivation.112 Interestingly, most mutant channels associated with long QT syndrome 1 respond similarly to wild-type channels, suggesting that the disease-associated channels would be susceptible to activation.112 To our knowledge, the therapeutic benefit of L-364,373 has not been tested. The gene discussed is KCNQ1; the disease is Prolonged QT interval.