NamePotassium voltage-gated channel subfamily KQT member 4
Synonyms
  • KQT-like 4
  • Potassium channel subunit alpha KvLQT4
  • Voltage-gated potassium channel subunit Kv7.4
Gene NameKCNQ4
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037202|Potassium voltage-gated channel subfamily KQT member 4
MAEAPPRRLGLGPPPGDAPRAELVALTAVQSEQGEAGGGGSPRRLGLLGSPLPPGAPLPG
PGSGSGSACGQRSSAAHKRYRRLQNWVYNVLERPRGWAFVYHVFIFLLVFSCLVLSVLST
IQEHQELANECLLILEFVMIVVFGLEYIVRVWSAGCCCRYRGWQGRFRFARKPFCVIDFI
VFVASVAVIAAGTQGNIFATSALRSMRFLQILRMVRMDRRGGTWKLLGSVVYAHSKELIT
AWYIGFLVLIFASFLVYLAEKDANSDFSSYADSLWWGTITLTTIGYGDKTPHTWLGRVLA
AGFALLGISFFALPAGILGSGFALKVQEQHRQKHFEKRRMPAANLIQAAWRLYSTDMSRA
YLTATWYYYDSILPSFRELALLFEHVQRARNGGLRPLEVRRAPVPDGAPSRYPPVATCHR
PGSTSFCPGESSRMGIKDRIRMGSSQRRTGPSKQHLAPPTMPTSPSSEQVGEATSPTKVQ
KSWSFNDRTRFRASLRLKPRTSAEDAPSEEVAEEKSYQCELTVDDIMPAVKTVIRSIRIL
KFLVAKRKFKETLRPYDVKDVIEQYSAGHLDMLGRIKSLQTRVDQIVGRGPGDRKAREKG
DKGPSDAEVVDEISMMGRVVKVEKQVQSIEHKLDLLLGFYSRCLRSGTSASLGAVQVPLF
DPDITSDYHSPVDHEDISVSAQTLSISRSVSTNMD
Number of residues695
Molecular Weight77099.99
Theoretical pI9.95
GO Classification
Functions
  • delayed rectifier potassium channel activity
  • potassium channel activity
Processes
  • sensory perception of sound
  • potassium ion transmembrane transport
  • inner ear morphogenesis
  • potassium ion transport
  • synaptic transmission
Components
  • basal plasma membrane
  • plasma membrane
  • voltage-gated potassium channel complex
  • integral component of membrane
General FunctionPotassium channel activity
Specific FunctionProbably important in the regulation of neuronal excitability. May underlie a potassium current involved in regulating the excitability of sensory cells of the cochlea. KCNQ4 channels are blocked by linopirdin, XE991 and bepridil, whereas clofilium is without significant effect. Muscarinic agonist oxotremorine-M strongly suppress KCNQ4 current in CHO cells in which cloned KCNQ4 channels were coexpressed with M1 muscarinic receptors.
Pfam Domain Function
Transmembrane Regions98-118 132-152 173-193 202-224 238-258 297-317
GenBank Protein IDNot Available
UniProtKB IDP56696
UniProtKB Entry NameKCNQ4_HUMAN
Cellular LocationBasal cell membrane
Gene sequence
>lcl|BSEQ0011718|Potassium voltage-gated channel subfamily KQT member 4 (KCNQ4)
ATGGCCGAGGCCCCCCCGCGCCGCCTCGGCCTGGGTCCCCCGCCCGGGGACGCCCCCCGC
GCGGAGCTAGTGGCGCTCACGGCCGTGCAGAGCGAACAGGGCGAGGCGGGCGGGGGCGGC
TCCCCGCGCCGCCTCGGCCTCCTGGGCAGCCCCCTGCCGCCGGGCGCGCCCCTCCCTGGG
CCGGGCTCCGGCTCGGGCTCCGCCTGCGGCCAGCGCTCCTCGGCCGCGCACAAGCGCTAC
CGCCGCCTGCAGAACTGGGTCTACAACGTGCTGGAGCGGCCCCGCGGCTGGGCCTTCGTC
TACCACGTCTTCATATTTTTGCTGGTCTTCAGCTGCCTGGTGCTGTCTGTGCTGTCCACT
ATCCAGGAGCACCAGGAACTTGCCAACGAGTGTCTCCTCATCTTGGAATTCGTGATGATC
GTGGTTTTCGGCTTGGAGTACATCGTCCGGGTCTGGTCCGCCGGATGCTGCTGCCGCTAC
CGAGGATGGCAGGGTCGCTTCCGCTTTGCCAGAAAGCCCTTCTGTGTCATCGACTTCATC
GTGTTCGTGGCCTCGGTGGCCGTCATCGCCGCGGGTACCCAGGGCAACATCTTCGCCACG
TCCGCGCTGCGCAGCATGCGCTTCCTGCAGATCCTGCGCATGGTGCGCATGGACCGCCGC
GGCGGCACCTGGAAGCTGCTGGGCTCAGTGGTCTACGCGCATAGCAAGGAGCTGATCACC
GCCTGGTACATCGGGTTCCTGGTGCTCATCTTCGCCTCCTTCCTGGTCTACCTGGCTGAG
AAGGACGCCAACTCCGACTTCTCCTCCTACGCCGACTCGCTCTGGTGGGGGACGATTACA
TTGACAACCATCGGCTATGGTGACAAGACACCGCACACATGGCTGGGCAGGGTCCTGGCT
GCTGGCTTCGCCTTACTGGGCATCTCTTTCTTTGCCCTGCCTGCCGGCATCCTAGGCTCC
GGCTTTGCCCTGAAGGTCCAGGAGCAGCACCGGCAGAAGCACTTCGAGAAGCGGAGGATG
CCGGCAGCCAACCTCATCCAGGCTGCCTGGCGCCTGTACTCCACCGATATGAGCCGGGCC
TACCTGACAGCCACCTGGTACTACTATGACAGTATCCTCCCATCCTTCAGAGAGCTGGCC
CTCTTGTTTGAGCACGTGCAACGGGCCCGCAATGGGGGCCTACGGCCCCTGGAGGTGCGG
CGGGCGCCGGTACCCGACGGAGCACCCTCCCGTTACCCGCCCGTTGCCACCTGCCACCGG
CCGGGCAGCACCTCCTTCTGCCCTGGGGAAAGCAGCCGGATGGGCATCAAAGACCGCATC
CGCATGGGCAGCTCCCAGCGGCGGACGGGTCCTTCCAAGCAGCATCTGGCACCTCCAACA
ATGCCCACCTCCCCAAGCAGCGAGCAGGTGGGTGAGGCCACCAGCCCCACCAAGGTGCAA
AAGAGCTGGAGCTTCAATGACCGCACCCGCTTCCGGGCATCTCTGAGACTCAAACCCCGC
ACCTCTGCTGAGGATGCCCCCTCAGAGGAAGTAGCAGAGGAGAAGAGCTACCAGTGTGAG
CTCACGGTGGACGACATCATGCCTGCTGTGAAGACAGTCATCCGCTCCATCAGGATTCTC
AAGTTCCTGGTGGCCAAAAGGAAATTCAAGGAGACACTGCGACCGTACGACGTGAAGGAC
GTCATTGAGCAGTACTCAGCAGGCCACCTGGACATGCTGGGCCGGATCAAGAGCCTGCAA
ACTCGGGTGGACCAAATTGTGGGTCGGGGGCCCGGGGACAGGAAGGCCCGGGAGAAGGGC
GACAAGGGGCCCTCCGACGCGGAGGTGGTGGATGAAATCAGCATGATGGGACGCGTGGTC
AAGGTGGAGAAGCAGGTGCAGTCCATCGAGCACAAGCTGGACCTGCTGTTGGGCTTCTAT
TCGCGCTGCCTGCGCTCTGGCACCTCGGCCAGCCTGGGCGCCGTGCAAGTGCCGCTGTTC
GACCCCGACATCACCTCCGACTACCACAGCCCTGTGGACCACGAGGACATCTCCGTCTCC
GCACAGACGCTCAGCATCTCCCGCTCGGTCAGCACCAACATGGACTGA
GenBank Gene IDAF105202
GeneCard IDNot Available
GenAtlas IDKCNQ4
HGNC IDHGNC:6298
Chromosome Location1
Locus1p34
References
  1. Kubisch C, Schroeder BC, Friedrich T, Lutjohann B, El-Amraoui A, Marlin S, Petit C, Jentsch TJ: KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness. Cell. 1999 Feb 5;96(3):437-46. 10025409
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  3. Selyanko AA, Hadley JK, Wood IC, Abogadie FC, Jentsch TJ, Brown DA: Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors. J Physiol. 2000 Feb 1;522 Pt 3:349-55. 10713961
  4. Sogaard R, Ljungstrom T, Pedersen KA, Olesen SP, Jensen BS: KCNQ4 channels expressed in mammalian cells: functional characteristics and pharmacology. Am J Physiol Cell Physiol. 2001 Apr;280(4):C859-66. 11245603
  5. Howard RJ, Clark KA, Holton JM, Minor DL Jr: Structural insight into KCNQ (Kv7) channel assembly and channelopathy. Neuron. 2007 Mar 1;53(5):663-75. 17329207
  6. Coucke PJ, Van Hauwe P, Kelley PM, Kunst H, Schatteman I, Van Velzen D, Meyers J, Ensink RJ, Verstreken M, Declau F, Marres H, Kastury K, Bhasin S, McGuirt WT, Smith RJ, Cremers CW, Van de Heyning P, Willems PJ, Smith SD, Van Camp G: Mutations in the KCNQ4 gene are responsible for autosomal dominant deafness in four DFNA2 families. Hum Mol Genet. 1999 Jul;8(7):1321-8. 10369879
  7. Talebizadeh Z, Kelley PM, Askew JW, Beisel KW, Smith SD: Novel mutation in the KCNQ4 gene in a large kindred with dominant progressive hearing loss. Hum Mutat. 1999;14(6):493-501. 10571947
  8. Van Hauwe P, Coucke PJ, Ensink RJ, Huygen P, Cremers CW, Van Camp G: Mutations in the KCNQ4 K+ channel gene, responsible for autosomal dominant hearing loss, cluster in the channel pore region. Am J Med Genet. 2000 Jul 31;93(3):184-7. 10925378
  9. Arnett J, Emery SB, Kim TB, Boerst AK, Lee K, Leal SM, Lesperance MM: Autosomal dominant progressive sensorineural hearing loss due to a novel mutation in the KCNQ4 gene. Arch Otolaryngol Head Neck Surg. 2011 Jan;137(1):54-9. doi: 10.1001/archoto.2010.234. 21242547