Potassium voltage-gated channel subfamily A member 1 (Kv1.1) (Kv1.1)

Potassium voltage-gated channel subfamily A member 1 (Kv1.1) is a transmembrane protein encoded by the KCNA1 gene that forms voltage-sensitive potassium channels, predominantly in the central and peripheral nervous systems (UniProt: P14625). It is essential for maintaining the resting membrane potential and regulating the frequency and shape of action potentials by facilitating potassium ion efflux during repolarization (PubMed: 10551865). Genetic mutations in KCNA1 are the primary cause of Episodic Ataxia Type 1 (EA1), a neurological disorder characterized by paroxysmal ataxia and continuous muscle twitching known as myokymia (NIH GeneReviews: NBK1221). In clinical practice, Kv1.1 is a target for the drug dalfampridine (4-aminopyridine), which blocks the channel to improve nerve conduction in patients with multiple sclerosis (StatPearls: NBK493195). Beyond the nervous system, Kv1.1 is also expressed in the distal convoluted tubule of the kidney, where it influences magnesium reabsorption, linking its dysfunction to familial hypomagnesemia (PubMed: 19164854).

Other names

KCNA1Voltage-gated potassium channel subunit Kv1.1RBK1HUK1MBK1AEMKEA1

Pharmacological agents typically act as pore blockers that inhibit the outward flow of potassium ions, leading to a prolongation of the action potential and increased neurotransmitter release at synaptic terminals (StatPearls: NBK493195).