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Kv1.1 Mediates the voltage-dependent potassium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a potassium-selective channel through which potassium ions may pass in accordance with their electrochemical gradient. Defects in KCNA1 are the cause of episodic ataxia type 1 (EA1); also known as paroxysmal or episodic ataxia with myokymia (EAM) or paroxysmal ataxia with neuromyotonia. EA1 is an autosomal dominant disorder characterized by brief episodes of ataxia and dysarthria. Neurological examination during and between the attacks demonstrates spontaneous, repetitive discharges in the distal musculature (myokymia) that arise from peripheral nerve. Nystagmus is absent. Defects in KCNA1 are the cause of myokymia isolated type 1 (MK1). Myokymia is a condition characterized by spontaneous involuntary contraction of muscle fiber groups that can be observed as vermiform movement of the overlying skin. Electromyography typically shows continuous motor unit activity with spontaneous oligo- and multiplet-discharges of high intraburst frequency (myokymic discharges). Isolated spontaneous muscle twitches occur in many persons and have no grave significance. Belongs to the potassium channel family. A (Shaker) (TC 1.A.1.2) subfamily. Kv1.1/KCNA1 sub-subfamily. Note: This description may include information from UniProtKB.
Protein type: Channel, potassium; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 12p13.32
Cellular Component: apical plasma membrane; axon terminus; cell junction; cell soma; cell surface; cytoplasmic vesicle; cytosol; dendrite; endoplasmic reticulum; integral component of plasma membrane; paranode region of axon; perikaryon; plasma membrane; presynaptic membrane; synapse; voltage-gated potassium channel complex
Molecular Function: delayed rectifier potassium channel activity; potassium channel activity; potassium ion transmembrane transporter activity; protein binding; voltage-gated potassium channel activity
Biological Process: detection of mechanical stimulus involved in sensory perception of pain; detection of mechanical stimulus involved in sensory perception of touch; generation of action potential; hippocampus development; neuroblast proliferation; neuromuscular process; potassium ion transport; protein homooligomerization; regulation of membrane potential; regulation of muscle contraction; startle response; synaptic transmission
Disease: Episodic Ataxia, Type 1
Reference #:  Q09470 (UniProtKB)
Alt. Names/Synonyms: AEMK; EA1; HBK1; HUK1; KCNA1; KV1.1; MBK1; MGC126782; MGC138385; MK1; Potassium voltage-gated channel subfamily A member 1; potassium voltage-gated channel, shaker-related subfamily, member 1 (episodic ataxia with myokymia); RBK1; Voltage-gated K(+) channel HuKI; Voltage-gated potassium channel HBK1; Voltage-gated potassium channel subunit Kv1.1
Gene Symbols: KCNA1
Molecular weight: 56,466 Da
Basal Isoelectric point: 5.08  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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