KCNC1 encodes Kv3.1, a voltage-gated potassium channel that plays a crucial role in neuronal excitability and rapid repolarization of fast-firing neurons 1. The channel functions as a delayed rectifier, opening in response to membrane voltage changes and allowing potassium ion efflux according to electrochemical gradients 1. KCNC1 forms homotetrameric or heterotetrameric channel complexes and is abundantly expressed in fast-spiking principal neurons and GABAergic interneurons throughout the auditory pathway and various brain regions 1. Disease associations include progressive myoclonus epilepsy type 7, with specific variants causing different phenotypes 2. The recurrent p.Ala421Val variant causes developmental and epileptic encephalopathy through loss of function without dominant-negative effects, while other loss-of-function variants can cause developmental encephalopathy without seizures 2. Notably, nonsense variants like p.Arg339* result in intellectual disability without epilepsy 3. The channel is sensitive to oxidative stress, which reduces its surface trafficking and current density - effects that can be reversed by melatonin treatment 1. KCNC1 has also been implicated in opioid use disorder through genome-wide association studies 4. The gene maps to chromosome 11 and shows high evolutionary conservation across mammals 5.