NamePotassium voltage-gated channel subfamily D member 3
Synonyms
  • Voltage-gated potassium channel subunit Kv4.3
Gene NameKCND3
OrganismHuman
Amino acid sequence
>lcl|BSEQ0006934|Potassium voltage-gated channel subfamily D member 3
MAAGVAAWLPFARAAAIGWMPVANCPMPLAPADKNKRQDELIVLNVSGRRFQTWRTTLER
YPDTLLGSTEKEFFFNEDTKEYFFDRDPEVFRCVLNFYRTGKLHYPRYECISAYDDELAF
YGILPEIIGDCCYEEYKDRKRENAERLMDDNDSENNQESMPSLSFRQTMWRAFENPHTST
LALVFYYVTGFFIAVSVITNVVETVPCGTVPGSKELPCGERYSVAFFCLDTACVMIFTVE
YLLRLFAAPSRYRFIRSVMSIIDVVAIMPYYIGLVMTNNEDVSGAFVTLRVFRVFRIFKF
SRHSQGLRILGYTLKSCASELGFLLFSLTMAIIIFATVMFYAEKGSSASKFTSIPASFWY
TIVTMTTLGYGDMVPKTIAGKIFGSICSLSGVLVIALPVPVIVSNFSRIYHQNQRADKRR
AQKKARLARIRVAKTGSSNAYLHSKRNGLLNEALELTGTPEEEHMGKTTSLIESQHHHLL
HCLEKTTGLSYLVDDPLLSVRTSTIKNHEFIDEQMFEQNCMESSMQNYPSTRSPSLSSHP
GLTTTCCSRRSKKTTHLPNSNLPATRLRSMQELSTIHIQGSEQPSLTTSRSSLNLKADDG
LRPNCKTSQITTAIISIPTPPALTPEGESRPPPASPGPNTNIPSIASNVVKVSAL
Number of residues655
Molecular Weight73450.53
Theoretical pI8.32
GO Classification
Functions
  • metal ion binding
  • ion channel binding
  • A-type (transient outward) potassium channel activity
Processes
  • potassium ion transport
  • synaptic transmission
  • protein homooligomerization
  • membrane repolarization
  • potassium ion export
Components
  • plasma membrane
  • voltage-gated potassium channel complex
  • integral component of membrane
  • dendrite
  • sarcolemma
General FunctionMetal ion binding
Specific FunctionPore-forming (alpha) subunit of voltage-gated rapidly inactivating A-type potassium channels. May contribute to I(To) current in heart and I(Sa) current in neurons. Channel properties are modulated by interactions with other alpha subunits and with regulatory subunits.
Pfam Domain Function
Transmembrane Regions182-202 222-242 257-277 287-307 321-341 382-402
GenBank Protein ID2935434
UniProtKB IDQ9UK17
UniProtKB Entry NameKCND3_HUMAN
Cellular LocationCell membrane
Gene sequence
>lcl|BSEQ0012506|Potassium voltage-gated channel subfamily D member 3 (KCND3)
ATGGCGGCCGGAGTTGCGGCCTGGCTGCCTTTTGCCCGGGCTGCGGCCATCGGGTGGATG
CCGGTGGCCAACTGCCCCATGCCCCTGGCCCCGGCCGACAAGAACAAGCGGCAGGATGAG
CTGATTGTCCTCAACGTGAGTGGGCGGAGGTTCCAGACCTGGAGGACCACGCTGGAGCGC
TACCCGGACACCCTGCTGGGCAGCACGGAGAAGGAGTTCTTCTTCAACGAGGACACCAAG
GAGTACTTCTTCGACCGGGACCCCGAGGTGTTCCGCTGCGTGCTCAACTTCTACCGCACG
GGGAAGCTGCACTACCCGCGCTACGAGTGCATCTCTGCCTACGACGACGAGCTGGCCTTC
TACGGCATCCTCCCGGAGATCATCGGGGACTGCTGCTACGAGGAGTACAAGGACCGCAAG
AGGGAGAACGCCGAGCGGCTCATGGACGACAACGACTCGGAGAACAACCAGGAGTCCATG
CCCTCGCTCAGCTTCCGCCAGACCATGTGGCGGGCCTTCGAGAACCCCCACACCAGCACG
CTGGCCCTGGTCTTCTACTACGTGACTGGCTTCTTCATCGCTGTCTCGGTCATCACCAAC
GTGGTGGAGACGGTGCCGTGCGGCACGGTCCCGGGCAGCAAGGAGCTGCCGTGCGGGGAG
CGCTACTCGGTGGCCTTCTTCTGCCTGGACACGGCGTGCGTCATGATCTTCACCGTGGAG
TACCTCCTGCGGCTCTTCGCGGCTCCCAGCCGCTACCGCTTCATCCGCAGCGTCATGAGC
ATCATCGACGTGGTGGCCATCATGCCCTACTACATCGGTCTGGTCATGACCAACAACGAG
GACGTGTCCGGCGCCTTCGTCACGCTCCGGGTCTTCCGCGTCTTCAGGATCTTCAAGTTT
TCCCGCCACTCCCAGGGCCTGCGGATCCTGGGCTACACACTGAAGAGCTGTGCCTCCGAA
CTGGGCTTTCTTCTCTTCTCCCTCACCATGGCCATCATCATCTTTGCCACTGTGATGTTT
TATGCCGAGAAGGGCTCCTCGGCCAGCAAGTTCACAAGCATCCCTGCCTCGTTTTGGTAC
ACCATTGTCACCATGACCACACTGGGATACGGAGACATGGTGCCTAAGACGATTGCAGGG
AAGATCTTCGGCTCCATCTGCTCCTTGAGTGGCGTCCTGGTCATTGCCCTGCCAGTCCCT
GTGATTGTTTCCAACTTTAGCCGGATTTACCACCAGAATCAGAGAGCTGATAAACGCAGG
GCACAAAAGAAGGCCCGCCTTGCCAGGATCCGTGTGGCCAAAACAGGCAGTTCGAATGCA
TACCTGCACAGCAAGCGCAACGGGCTCCTCAACGAGGCGCTGGAGCTGACGGGCACCCCA
GAAGAGGAGCACATGGGCAAGACCACCTCACTCATCGAGAGCCAGCATCATCACCTGCTG
CACTGCCTGGAAAAAACCACTGGGTTGTCCTATCTTGTGGATGATCCCCTGTTATCTGTA
CGAACCTCCACCATCAAGAACCACGAGTTTATTGATGAGCAGATGTTTGAGCAGAACTGC
ATGGAGAGTTCAATGCAGAACTACCCATCCACAAGAAGTCCCTCACTGTCCAGCCACCCA
GGCCTCACTACCACCTGCTGCTCCCGTCGTAGTAAGAAGACCACACACCTGCCCAATTCT
AACCTGCCAGCTACTCGCCTGCGCAGCATGCAAGAGCTCAGCACGATCCACATCCAGGGC
AGTGAGCAGCCCTCCCTCACAACCAGTCGCTCCAGCCTTAATTTGAAAGCAGACGACGGA
CTGAGACCAAACTGCAAAACATCCCAGATCACCACAGCCATCATCAGCATCCCCACTCCC
CCAGCGCTAACCCCAGAGGGGGAAAGTCGGCCACCCCCTGCCAGCCCAGGCCCCAACACG
AACATTCCTTCCATAGCCAGCAATGTTGTCAAGGTCTCCGCCTTGTAA
GenBank Gene IDAF048712
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:6239
Chromosome Location1
Locus1p13.3
References
  1. Kong W, Po S, Yamagishi T, Ashen MD, Stetten G, Tomaselli GF: Isolation and characterization of the human gene encoding Ito: further diversity by alternative mRNA splicing. Am J Physiol. 1998 Dec;275(6 Pt 2):H1963-70. 9843794
  2. Dilks D, Ling HP, Cockett M, Sokol P, Numann R: Cloning and expression of the human kv4.3 potassium channel. J Neurophysiol. 1999 Apr;81(4):1974-7. 10200233
  3. Isbrandt D, Leicher T, Waldschutz R, Zhu X, Luhmann U, Michel U, Sauter K, Pongs O: Gene structures and expression profiles of three human KCND (Kv4) potassium channels mediating A-type currents I(TO) and I(SA). Genomics. 2000 Mar 1;64(2):144-54. 10729221
  4. 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. 16710414
  5. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. 15489334
  6. Deschenes I, Tomaselli GF: Modulation of Kv4.3 current by accessory subunits. FEBS Lett. 2002 Sep 25;528(1-3):183-8. 12297301
  7. El-Haou S, Balse E, Neyroud N, Dilanian G, Gavillet B, Abriel H, Coulombe A, Jeromin A, Hatem SN: Kv4 potassium channels form a tripartite complex with the anchoring protein SAP97 and CaMKII in cardiac myocytes. Circ Res. 2009 Mar 27;104(6):758-69. doi: 10.1161/CIRCRESAHA.108.191007. Epub 2009 Feb 12. 19213956
  8. Giudicessi JR, Ye D, Tester DJ, Crotti L, Mugione A, Nesterenko VV, Albertson RM, Antzelevitch C, Schwartz PJ, Ackerman MJ: Transient outward current (I(to)) gain-of-function mutations in the KCND3-encoded Kv4.3 potassium channel and Brugada syndrome. Heart Rhythm. 2011 Jul;8(7):1024-32. doi: 10.1016/j.hrthm.2011.02.021. Epub 2011 Feb 22. 21349352
  9. Giudicessi JR, Ye D, Kritzberger CJ, Nesterenko VV, Tester DJ, Antzelevitch C, Ackerman MJ: Novel mutations in the KCND3-encoded Kv4.3 K+ channel associated with autopsy-negative sudden unexplained death. Hum Mutat. 2012 Jun;33(6):989-97. doi: 10.1002/humu.22058. Epub 2012 Mar 27. 22457051
  10. Scannevin RH, Wang K, Jow F, Megules J, Kopsco DC, Edris W, Carroll KC, Lu Q, Xu W, Xu Z, Katz AH, Olland S, Lin L, Taylor M, Stahl M, Malakian K, Somers W, Mosyak L, Bowlby MR, Chanda P, Rhodes KJ: Two N-terminal domains of Kv4 K(+) channels regulate binding to and modulation by KChIP1. Neuron. 2004 Feb 19;41(4):587-98. 14980207
  11. Wang H, Yan Y, Liu Q, Huang Y, Shen Y, Chen L, Chen Y, Yang Q, Hao Q, Wang K, Chai J: Structural basis for modulation of Kv4 K+ channels by auxiliary KChIP subunits. Nat Neurosci. 2007 Jan;10(1):32-9. Epub 2006 Dec 24. 17187064
  12. Sjoblom T, Jones S, Wood LD, Parsons DW, Lin J, Barber TD, Mandelker D, Leary RJ, Ptak J, Silliman N, Szabo S, Buckhaults P, Farrell C, Meeh P, Markowitz SD, Willis J, Dawson D, Willson JK, Gazdar AF, Hartigan J, Wu L, Liu C, Parmigiani G, Park BH, Bachman KE, Papadopoulos N, Vogelstein B, Kinzler KW, Velculescu VE: The consensus coding sequences of human breast and colorectal cancers. Science. 2006 Oct 13;314(5797):268-74. Epub 2006 Sep 7. 16959974
  13. Lee YC, Durr A, Majczenko K, Huang YH, Liu YC, Lien CC, Tsai PC, Ichikawa Y, Goto J, Monin ML, Li JZ, Chung MY, Mundwiller E, Shakkottai V, Liu TT, Tesson C, Lu YC, Brice A, Tsuji S, Burmeister M, Stevanin G, Soong BW: Mutations in KCND3 cause spinocerebellar ataxia type 22. Ann Neurol. 2012 Dec;72(6):859-69. doi: 10.1002/ana.23701. 23280837
  14. Duarri A, Jezierska J, Fokkens M, Meijer M, Schelhaas HJ, den Dunnen WF, van Dijk F, Verschuuren-Bemelmans C, Hageman G, van de Vlies P, Kusters B, van de Warrenburg BP, Kremer B, Wijmenga C, Sinke RJ, Swertz MA, Kampinga HH, Boddeke E, Verbeek DS: Mutations in potassium channel kcnd3 cause spinocerebellar ataxia type 19. Ann Neurol. 2012 Dec;72(6):870-80. doi: 10.1002/ana.23700. 23280838