Potassium voltage-gated channel subfamily A member 4

UniProt P22459 Organism Homo sapiens Gene KCNA4, KCNA4L

Also known as: KCNA4, KCNA4L

Function

Voltage-gated potassium channel that mediates transmembrane potassium transport in excitable membranes. Forms tetrameric potassium-selective channels through which potassium ions pass in accordance with their electrochemical gradient. The channel alternates between opened and closed conformations in response to the voltage difference across the membrane. Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNA1, KCNA2, KCNA4, KCNA5, and possibly other family members as well; channel properties depend on the type of alpha subunits that are part of the channel. Channel properties are modulated by cytoplasmic beta subunits that regulate the subcellular location of the alpha subunits and promote rapid inactivation. In vivo, membranes probably contain a mixture of heteromeric potassium channel complexes, making it difficult to assign currents observed in intact tissues to any particular potassium channel family member. Homotetrameric KCNA4 forms a potassium channel that opens in response to membrane depolarization, followed by rapid spontaneous channel closure. Likewise, a heterotetrameric channel formed by KCNA1 and KCNA4 shows rapid inactivation.

Classification

Family (Pfam): PF02214 BTB_2, PF00520 Ion_trans, PF07941 K_channel_TID
InterPro: BTB/POZ_dom, Ion_trans_dom, K_chnl_volt-dep_Kv, K_chnl_volt-dep_Kv1, K_chnl_volt-dep_Kv1.4, K_chnl_volt-dep_Kv1.4_TID, K_chnl_volt-dep_Kv1.4_TID_sf, SKP1/BTB/POZ_sf, T1-type_BTB, VG_K_chnl, Volt_channel_dom_sf
Functional cluster: Homeobox & Zinc-Finger Transcription Factors

Gene Ontology · 14

Disease associations

microcephaly, cataracts, impaired intellectual development, and dystonia with abnormal striatum MONDO:0032656

Drugs targeting this protein · 6

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Potassium voltage-gated channel subfamily A member 4 1 shared papers
Potassium channel regulatory protein 1 shared papers
Potassium voltage-gated channel subfamily A member 5 1 shared papers
Potassium voltage-gated channel subfamily A member 1 3 shared papers
Voltage-gated potassium channel KCNC4 1 shared papers
Potassium voltage-gated channel subfamily A member 4 2 shared papers
Potassium voltage-gated channel subfamily A member 2 2 shared papers
Voltage-gated potassium channel subunit beta-1 1 shared papers
Potassium voltage-gated channel subfamily A member 2 1 shared papers

Literature · 9 cited papers

KCNA4 deficiency leads to a syndrome of abnormal striatum, congenital cataract and intellectual disability. J. Med. Genet. · 2016
Potassium channel regulator KCNRG regulates surface expression of Shaker-type potassium channels. Biochem. Biophys. Res. Commun. · 2010
Human potassium channel genes: molecular cloning and functional expression. Mol. Cell. Neurosci. · 1990
Episodic ataxia type 1 mutations in the KCNA1 gene impair the fast inactivation properties of the human potassium channels Kv1.4-1.1/Kvbeta1.1 and Kv1.4-1.1/Kvbeta1.2. Eur. J. Neurosci. · 2006
Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature · 2006
NMR structure of inactivation gates from mammalian voltage-dependent potassium channels. Nature · 1997
Heteromultimeric assembly of human potassium channels. Molecular basis of a transient outward current? Circ. Res. · 1993
Sequence of a human fetal skeletal muscle potassium channel cDNA related to RCK4. Nucleic Acids Res. · 1990
Molecular cloning and characterization of two voltage-gated K+ channel cDNAs from human ventricle. FASEB J. · 1991

A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.