K+ voltage-gated channel subfamily A member 1; voltage-gated K+ channel subunit Kv1.1; HUKI; HBK1 (KCNA1)

Function

• mediates voltage-dependent K+ permeability of excitable membranes
• assumes opened or closed conformation in response to the voltage difference across the membrane
• forms a K+-selective channel through which K+ may pass in accordance with their electrochemical gradient
• palmitoylated on Cys-243; which may be required for membrane targeting
• heterotetramer of K+ channel proteins
• binds KCNAB2 & PDZ domains of DLG1, DLG2 & DLG4 (putative)
• interacts with LGI1 within a complex containing LGI1, KCNA4 & KCNAB1 (putative)

Structure

• the N-terminus may be important in determining the rate of inactivation of the channel while the tail may play a role in modulation of channel activity &/or targeting of the channel to specific subcellular compartments
• the segment S4 is probably the voltage-sensor & is characterized by a series of positively charged amino acids at every third position
• belongs to the K+ channel family, A (Shaker) subfamily

Compartment

membrane

RNA editing

• partially edited
• RNA editing varies from 17% in the caudate nucleus to 68% in the spinal cord & to 77% in the medulla

Pathology

• defects in KCNA1 are the cause of:
  • episodic ataxia type 1
  • myokymia isolated type 1

More general terms

• voltage-gated K+ channel alpha subunit
• glycoprotein
• phosphoprotein

Database

• UniProt: http://www.uniprot.org/uniprot/Q09470.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3736
• OMIM: https://mirror.omim.org/entry/160120
• OMIM: https://mirror.omim.org/entry/176260