Key result
Treatment with the KCNQ opener pynegabine (HN37) dramatically ameliorated the increased susceptibility to evoked seizures in Kcnq2-R207W mutant mice.
Why the study?
Although mouse lines with Kcnq2 pore variants exist, no mouse line carrying Kcnq2 voltage-sensor mutations had been described to evaluate the physiological consequences of voltage sensor domain dysfunction.
The Kcnq2-R207W mouse model exhibits spontaneous and evoked seizures that are ameliorated by the KCNQ opener pynegabine, suggesting its potential for treating KCNQ2-related epilepsy.
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Hypothesis-generating for KCNQ openers in KCNQ2 epilepsy; leaves open translation to patients.
Tian et al. (2022) studied KCNQ2-related epilepsy (n=64). Pynegabine (HN37) vs. Untreated mutant mice was evaluated on Susceptibility to evoked seizures. Treatment with the KCNQ opener pynegabine (HN37) dramatically ameliorated the increased susceptibility to evoked seizures in Kcnq2-R207W mutant mice.