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Identification
HMDB Protein ID HMDBP10849
Secondary Accession Numbers
  • 17139
Name Potassium voltage-gated channel subfamily KQT member 4
Synonyms
  1. KQT-like 4
  2. Potassium channel subunit alpha KvLQT4
  3. Voltage-gated potassium channel subunit Kv7.4
Gene Name KCNQ4
Protein Type Unknown
Biological Properties
General Function Involved in ion channel activity
Specific Function Probably 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 muscarinnic receptors
Pathways Not Available
Reactions Not Available
GO Classification
Component
membrane
cell part
ion channel complex
cation channel complex
potassium channel complex
voltage-gated potassium channel complex
macromolecular complex
protein complex
Function
transmembrane transporter activity
substrate-specific transmembrane transporter activity
ion transmembrane transporter activity
transporter activity
ion channel activity
cation channel activity
potassium channel activity
voltage-gated potassium channel activity
Process
establishment of localization
transport
transmembrane transport
monovalent inorganic cation transport
potassium ion transport
ion transport
cation transport
Cellular Location
  1. Multi-pass membrane protein
  2. Basal cell membrane
Gene Properties
Chromosome Location Chromosome:1
Locus 1p34
SNPs KCNQ4
Gene Sequence
>2088 bp
ATGGCCGAGGCCCCCCCGCGCCGCCTCGGCCTGGGTCCCCCGCCCGGGGACGCCCCCCGC
GCGGAGCTAGTGGCGCTCACGGCCGTGCAGAGCGAACAGGGCGAGGCGGGCGGGGGCGGC
TCCCCGCGCCGCCTCGGCCTCCTGGGCAGCCCCCTGCCGCCGGGCGCGCCCCTCCCTGGG
CCGGGCTCCGGCTCGGGCTCCGCCTGCGGCCAGCGCTCCTCGGCCGCGCACAAGCGCTAC
CGCCGCCTGCAGAACTGGGTCTACAACGTGCTGGAGCGGCCCCGCGGCTGGGCCTTCGTC
TACCACGTCTTCATATTTTTGCTGGTCTTCAGCTGCCTGGTGCTGTCTGTGCTGTCCACT
ATCCAGGAGCACCAGGAACTTGCCAACGAGTGTCTCCTCATCTTGGAATTCGTGATGATC
GTGGTTTTCGGCTTGGAGTACATCGTCCGGGTCTGGTCCGCCGGATGCTGCTGCCGCTAC
CGAGGATGGCAGGGTCGCTTCCGCTTTGCCAGAAAGCCCTTCTGTGTCATCGACTTCATC
GTGTTCGTGGCCTCGGTGGCCGTCATCGCCGCGGGTACCCAGGGCAACATCTTCGCCACG
TCCGCGCTGCGCAGCATGCGCTTCCTGCAGATCCTGCGCATGGTGCGCATGGACCGCCGC
GGCGGCACCTGGAAGCTGCTGGGCTCAGTGGTCTACGCGCATAGCAAGGAGCTGATCACC
GCCTGGTACATCGGGTTCCTGGTGCTCATCTTCGCCTCCTTCCTGGTCTACCTGGCCGAG
AAGGACGCCAACTCCGACTTCTCCTCCTACGCCGACTCGCTCTGGTGGGGGACGATTACA
TTGACAACCATCGGCTATGGTGACAAGACACCGCACACATGGCTGGGCAGGGTCCTGGCT
GCTGGCTTCGCCTTACTGGGCATCTCTTTCTTTGCCCTGCCTGCCGGCATCCTAGGCTCC
GGCTTTGCCCTGAAGGTCCAGGAGCAGCACCGGCAGAAGCACTTCGAGAAGCGGAGGATG
CCGGCAGCCAACCTCATCCAGGCTGCCTGGCGCCTGTACTCCACCGATATGAGCCGGGCC
TACCTGACAGCCACCTGGTACTACTATGACAGTATCCTCCCATCCTTCAGAGAGCTGGCC
CTCTTGTTTGAGCACGTGCAACGGGCCCGCAATGGGGGCCTACGGCCCCTGGAGGTGCGG
CGGGCGCCGGTACCCGACGGAGCACCCTCCCGTTACCCGCCCGTTGCCACCTGCCACCGG
CCGGGCAGCACCTCCTTCTGCCCTGGGGAAAGCAGCCGGATGGGCATCAAAGACCGCATC
CGCATGGGCAGCTCCCAGCGGCGGACGGGTCCTTCCAAGCAGCAGCTGGCACCTCCAACA
ATGCCCACCTCCCCAAGCAGCGAGCAGGTGGGTGAGGCCACCAGCCCCACCAAGGTGCAA
AAGAGCTGGAGCTTCAATGACCGCACCCGCTTCCGGGCATCTCTGAGACTCAAACCCCGC
ACCTCTGCTGAGGATGCCCCCTCAGAGGAAGTAGCAGAGGAGAAGAGCTACCAGTGTGAG
CTCACGGTGGACGACATCATGCCTGCTGTGAAGACAGTCATCCGCTCCATCAGGATTCTC
AAGTTCCTGGTGGCCAAAAGGAAATTCAAGGAGACACTGCGACCGTACGACGTGAAGGAC
GTCATTGAGCAGTACTCAGCAGGCCACCTGGACATGCTGGGCCGGATCAAGAGCCTGCAA
ACTCGGGTGGACCAAATTGTGGGTCGGGGGCCCGGGGACAGGAAGGCCCGGGAGAAGGGC
GACAAGGGGCCCTCCGACGCGGAGGTGGTGGATGAAATCAGCATGATGGGACGCGTGGTC
AAGGTGGAGAAGCAGGTGCAGTCCATCGAGCACAAGCTGGACCTGCTGTTGGGCTTCTAT
TCGCGCTGCCTGCGCTCTGGCACCTCGGCCAGCCTGGGCGCCGTGCAAGTGCCGCTGTTC
GACCCCGACATCACCTCCGACTACCACAGCCCTGTGGACCACGAGGACATCTCCGTCTCC
GCACAGACGCTCAGCATCTCCCGCTCGGTCAGCACCAACATGGACTGA
Protein Properties
Number of Residues 695
Molecular Weight 77100.0
Theoretical pI 9.95
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • 98-118
  • 132-152
  • 173-193
  • 202-224
  • 238-258
  • 297-317
Protein Sequence
>Potassium voltage-gated channel subfamily KQT member 4
MAEAPPRRLGLGPPPGDAPRAELVALTAVQSEQGEAGGGGSPRRLGLLGSPLPPGAPLPG
PGSGSGSACGQRSSAAHKRYRRLQNWVYNVLERPRGWAFVYHVFIFLLVFSCLVLSVLST
IQEHQELANECLLILEFVMIVVFGLEYIVRVWSAGCCCRYRGWQGRFRFARKPFCVIDFI
VFVASVAVIAAGTQGNIFATSALRSMRFLQILRMVRMDRRGGTWKLLGSVVYAHSKELIT
AWYIGFLVLIFASFLVYLAEKDANSDFSSYADSLWWGTITLTTIGYGDKTPHTWLGRVLA
AGFALLGISFFALPAGILGSGFALKVQEQHRQKHFEKRRMPAANLIQAAWRLYSTDMSRA
YLTATWYYYDSILPSFRELALLFEHVQRARNGGLRPLEVRRAPVPDGAPSRYPPVATCHR
PGSTSFCPGESSRMGIKDRIRMGSSQRRTGPSKQHLAPPTMPTSPSSEQVGEATSPTKVQ
KSWSFNDRTRFRASLRLKPRTSAEDAPSEEVAEEKSYQCELTVDDIMPAVKTVIRSIRIL
KFLVAKRKFKETLRPYDVKDVIEQYSAGHLDMLGRIKSLQTRVDQIVGRGPGDRKAREKG
DKGPSDAEVVDEISMMGRVVKVEKQVQSIEHKLDLLLGFYSRCLRSGTSASLGAVQVPLF
DPDITSDYHSPVDHEDISVSAQTLSISRSVSTNMD
GenBank ID Protein 4262523
UniProtKB/Swiss-Prot ID P56696
UniProtKB/Swiss-Prot Entry Name KCNQ4_HUMAN
PDB IDs Not Available
GenBank Gene ID AF105202
GeneCard ID KCNQ4
GenAtlas ID KCNQ4
HGNC ID HGNC:6298
References
General References
  1. Gregory SG, Barlow KF, McLay KE, Kaul R, Swarbreck D, Dunham A, Scott CE, Howe KL, Woodfine K, Spencer CC, Jones MC, Gillson C, Searle S, Zhou Y, Kokocinski F, McDonald L, Evans R, Phillips K, Atkinson A, Cooper R, Jones C, Hall RE, Andrews TD, Lloyd C, Ainscough R, Almeida JP, Ambrose KD, Anderson F, Andrew RW, Ashwell RI, Aubin K, Babbage AK, Bagguley CL, Bailey J, Beasley H, Bethel G, Bird CP, Bray-Allen S, Brown JY, Brown AJ, Buckley D, Burton J, Bye J, Carder C, Chapman JC, Clark SY, Clarke G, Clee C, Cobley V, Collier RE, Corby N, Coville GJ, Davies J, Deadman R, Dunn M, Earthrowl M, Ellington AG, Errington H, Frankish A, Frankland J, French L, Garner P, Garnett J, Gay L, Ghori MR, Gibson R, Gilby LM, Gillett W, Glithero RJ, Grafham DV, Griffiths C, Griffiths-Jones S, Grocock R, Hammond S, Harrison ES, Hart E, Haugen E, Heath PD, Holmes S, Holt K, Howden PJ, Hunt AR, Hunt SE, Hunter G, Isherwood J, James R, Johnson C, Johnson D, Joy A, Kay M, Kershaw JK, Kibukawa M, Kimberley AM, King A, Knights AJ, Lad H, Laird G, Lawlor S, Leongamornlert DA, Lloyd DM, Loveland J, Lovell J, Lush MJ, Lyne R, Martin S, Mashreghi-Mohammadi M, Matthews L, Matthews NS, McLaren S, Milne S, Mistry S, Moore MJ, Nickerson T, O'Dell CN, Oliver K, Palmeiri A, Palmer SA, Parker A, Patel D, Pearce AV, Peck AI, Pelan S, Phelps K, Phillimore BJ, Plumb R, Rajan J, Raymond C, Rouse G, Saenphimmachak C, Sehra HK, Sheridan E, Shownkeen R, Sims S, Skuce CD, Smith M, Steward C, Subramanian S, Sycamore N, Tracey A, Tromans A, Van Helmond Z, Wall M, Wallis JM, White S, Whitehead SL, Wilkinson JE, Willey DL, Williams H, Wilming L, Wray PW, Wu Z, Coulson A, Vaudin M, Sulston JE, Durbin R, Hubbard T, Wooster R, Dunham I, Carter NP, McVean G, Ross MT, Harrow J, Olson MV, Beck S, Rogers J, Bentley DR, Banerjee R, Bryant SP, Burford DC, Burrill WD, Clegg SM, Dhami P, Dovey O, Faulkner LM, Gribble SM, Langford CF, Pandian RD, Porter KM, Prigmore E: The DNA sequence and biological annotation of human chromosome 1. Nature. 2006 May 18;441(7091):315-21. [PubMed:16710414 ]
  2. 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. [PubMed:10713961 ]
  3. 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. [PubMed:10025409 ]
  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. [PubMed:11245603 ]
  5. 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. [PubMed:10369879 ]
  6. 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. [PubMed:10571947 ]
  7. 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. [PubMed:10925378 ]