KCNC1

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Voltage-gated potassium (KCN) channels are a diverse group of channels and play a central role in neuronal excitability. They contribute to the determination of the resting membrane potential, shaping of action potentials, the modulation of transmitter release and pattern of action potential discharges, and the regulation of frequency. Channels formed by Shaw-like KCNC subunits activate rapidly at potentials positive to-10 mV.

KCNC1 encodes Kv3.1, which functions as a highly conserved potassium ion channel subunit of the Kv3 subfamily of voltage-gated tetrameric potassium ion channels, major determinants of high-frequency neuronal firing. Kv3.1 is expressed in cerebellar granule cells, the thalamic reticular nucleus, a subset of cells in cerebral cortex and hippocampus, and several brainstem nuclei involved in auditory signal processing. It appears that, with the exception of cerebellar granule cells, Kv3.1 channels are expressed in parvalbumin-containing, fast-spiking GABAergic neurons. Outside the central nervous system, Kv3.1 is expressed in skeletal muscle and in T-lymphocytes.

It has been reported that a recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy (PME). PME is one of the most destructive types of epilepsy. They are clinically and genetically heterogeneous, characterized by core features of action myoclonus, progressive neurological decline and tonic-clonic seizures. Most of the molecular characteristics of PMEs are inherited by autosomal recessive inheritance, with rare cases showing autosomal dominant or mitochondrial inheritance.

Computer simulations have suggested that the KCNC1 conductance improves postsynaptic temporal coding precisio by reducing the width of the action potential without compromising its amplitude. In the rat, alternative splicing of the KCNC1 gene gives rise to two proteins, KCNC1a and KCNC1b. Splicing varieties differ in their carboxyl termini. The in vivo expression of the two KCNC1 gene products in rat is temporally regulated during development such that KCNC1a is predominant in the embryonic and neonatal neurons, while levels of KCNC1b expression are up-regulated after postnatal day 10. In the chicken, a high threshold potassium current with kinetics similar to that of KCNC1 has been reported in developing neurons in vitro.