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Cryo-EM structure of the human KCNQ2/KCNQ3 heterotetramer (3:1 stoichiometry) with CLM142 and PIP2 in open state (M2223 open, without symmetry expansion)
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Structural highlights
DiseaseKCNQ3_HUMAN Benign familial infantile epilepsy;Benign familial neonatal seizures;Juvenile myoclonic epilepsy. The disease is caused by mutations affecting the gene represented in this entry. Defects in KCNQ3 may be involved in epileptic disorders. These are characterized by paroxysmal transient disturbances of the electrical activity of the brain that may be manifested as episodic impairment or loss of consciousness, abnormal motor phenomena, psychic or sensory disturbances, or perturbation of the autonomic nervous system.[1] FunctionKCNQ3_HUMAN Probably important in the regulation of neuronal excitability. Associates with KCNQ2 or KCNQ5 to form a potassium channel with essentially identical properties to the channel underlying the native M-current, a slowly activating and deactivating potassium conductance which plays a critical role in determining the subthreshold electrical excitability of neurons as well as the responsiveness to synaptic inputs. References
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This page was last modified 04:50, 13 August 2026.