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NamePotassium voltage-gated channel subfamily C member 3
Synonyms
  • KSHIIID
  • Voltage-gated potassium channel subunit Kv3.3
Gene NameKCNC3
OrganismHuman
Amino acid sequence
>lcl|BSEQ0019627|Potassium voltage-gated channel subfamily C member 3
MLSSVCVSSFRGRQGASKQQPAPPPQPPESPPPPPLPPQQQQPAQPGPAASPAGPPAPRG
PGDRRAEPCPGLPAAAMGRHGGGGGDSGKIVINVGGVRHETYRSTLRTLPGTRLAGLTEP
EAAARFDYDPGADEFFFDRHPGVFAYVLNYYRTGKLHCPADVCGPLFEEELGFWGIDETD
VEACCWMTYRQHRDAEEALDSFEAPDPAGAANAANAAGAHDGGLDDEAGAGGGGLDGAGG
ELKRLCFQDAGGGAGGPPGGAGGAGGTWWRRWQPRVWALFEDPYSSRAARYVAFASLFFI
LISITTFCLETHEGFIHISNKTVTQASPIPGAPPENITNVEVETEPFLTYVEGVCVVWFT
FEFLMRITFCPDKVEFLKSSLNIIDCVAILPFYLEVGLSGLSSKAAKDVLGFLRVVRFVR
ILRIFKLTRHFVGLRVLGHTLRASTNEFLLLIIFLALGVLIFATMIYYAERIGADPDDIL
GSNHTYFKNIPIGFWWAVVTMTTLGYGDMYPKTWSGMLVGALCALAGVLTIAMPVPVIVN
NFGMYYSLAMAKQKLPKKKNKHIPRPPQPGSPNYCKPDPPPPPPPHPHHGSGGISPPPPI
TPPSMGVTVAGAYPAGPHTHPGLLRGGAGGLGIMGLPPLPAPGEPCPLAQEEVIEINRAD
PRPNGDPAAAALAHEDCPAIDQPAMSPEDKSPITPGSRGRYSRDRACFLLTDYAPSPDGS
IRKATGAPPLPPQDWRKPGPPSFLPDLNANAAAWISP
Number of residues757
Molecular Weight80577.23
Theoretical pINot Available
GO Classification
Functions
  • delayed rectifier potassium channel activity
  • voltage-gated potassium channel activity
Processes
  • synaptic transmission
  • protein tetramerization
  • potassium ion transmembrane transport
  • protein homooligomerization
  • regulation of neurotransmitter secretion
Components
  • neuromuscular junction
  • neuronal cell body membrane
  • plasma membrane
  • voltage-gated potassium channel complex
  • integral component of membrane
  • axon terminus
  • axolemma
  • dendrite membrane
General FunctionVoltage-gated potassium channel activity
Specific FunctionThis protein mediates the voltage-dependent potassium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a potassium-selective channel through which potassium ions may pass in accordance with their electrochemical gradient.
Pfam Domain Function
Transmembrane Regions291-309 351-370 380-398 412-434 448-469 518-539
GenBank Protein IDNot Available
UniProtKB IDQ14003
UniProtKB Entry NameKCNC3_HUMAN
Cellular LocationCell membrane
Gene sequence
>lcl|BSEQ0019628|Potassium voltage-gated channel subfamily C member 3 (KCNC3)
ATGCTGAGCTCAGTCTGCGTCTCGTCCTTCCGCGGGCGCCAGGGGGCCAGCAAGCAGCAG
CCGGCGCCACCGCCGCAGCCGCCCGAGTCCCCGCCGCCGCCACCGCTGCCGCCGCAGCAG
CAGCAGCCTGCGCAGCCCGGCCCCGCCGCGTCCCCGGCGGGCCCCCCGGCACCCCGCGGG
CCCGGGGACCGGCGCGCCGAGCCATGCCCCGGGCTGCCGGCGGCGGCCATGGGGCGGCAC
GGCGGCGGCGGTGGCGACAGCGGCAAGATCGTGATCAACGTGGGCGGCGTGCGCCATGAG
ACGTACCGCTCGACGCTGCGCACCCTGCCGGGGACGCGGCTGGCCGGCCTGACGGAGCCC
GAGGCGGCGGCACGCTTCGACTACGACCCGGGCGCCGACGAGTTCTTCTTTGACCGGCAC
CCGGGAGTCTTCGCGTACGTGCTCAACTACTACCGCACCGGCAAGCTGCACTGCCCAGCC
GACGTGTGCGGGCCCCTGTTTGAGGAGGAGCTCGGCTTCTGGGGCATCGACGAGACCGAC
GTGGAGGCCTGCTGCTGGATGACCTACCGGCAGCATCGCGACGCTGAGGAGGCGCTCGAC
TCCTTCGAGGCGCCCGACCCCGCGGGCGCCGCCAACGCCGCCAACGCCGCAGGCGCCCAC
GACGGAGGCCTGGACGACGAGGCGGGCGCGGGCGGCGGCGGCCTGGACGGAGCGGGCGGC
GAGCTCAAGCGCCTCTGCTTCCAGGACGCGGGCGGCGGCGCCGGGGGGCCGCCAGGGGGC
GCGGGCGGCGCGGGCGGCACATGGTGGCGCCGCTGGCAGCCCCGCGTGTGGGCGCTCTTC
GAGGACCCCTACTCGTCGCGGGCTGCCAGGTATGTGGCCTTCGCCTCCCTCTTCTTCATC
CTCATCTCCATCACCACCTTCTGCCTGGAAACCCATGAGGGCTTCATCCATATTAGCAAC
AAGACGGTGACCCAGGCCTCCCCGATCCCCGGGGCACCTCCGGAGAACATCACCAACGTG
GAGGTGGAGACGGAGCCCTTCCTGACCTACGTGGAGGGGGTGTGCGTGGTCTGGTTCACC
TTCGAGTTCCTCATGCGCATCACCTTCTGCCCAGACAAGGTGGAGTTTCTTAAAAGCAGC
CTCAACATCATCGACTGTGTGGCCATCCTGCCCTTCTATCTCGAGGTGGGCCTCTCGGGC
CTCAGCTCCAAGGCCGCCAAAGACGTGCTGGGCTTCCTGCGGGTGGTCCGCTTCGTCCGC
ATCCTGCGCATCTTCAAGCTGACCCGGCACTTCGTGGGGCTGCGCGTGCTGGGACACACG
CTCCGCGCCAGCACCAACGAGTTCCTGCTGCTCATCATCTTCCTGGCCCTGGGGGTGCTC
ATCTTCGCCACCATGATTTACTACGCTGAGCGCATTGGCGCCGACCCCGATGACATCCTG
GGCTCCAACCACACCTACTTCAAGAACATCCCCATTGGCTTCTGGTGGGCTGTGGTCACC
ATGACGACCCTGGGCTATGGAGACATGTACCCCAAGACGTGGTCGGGGATGCTGGTCGGG
GCGCTGTGTGCCCTGGCGGGGGTGCTGACCATCGCCATGCCTGTGCCCGTCATTGTCAAC
AACTTTGGCATGTACTATTCGCTGGCCATGGCCAAGCAGAAGCTGCCCAAGAAGAAGAAC
AAACACATCCCCCGGCCCCCGCAACCGGGCTCGCCCAACTACTGCAAGCCTGACCCACCC
CCGCCACCCCCGCCCCACCCGCACCACGGCAGCGGGGGCATCAGCCCGCCGCCACCCATC
ACCCCACCCTCCATGGGGGTGACTGTGGCCGGGGCCTACCCAGCGGGGCCCCACACGCAC
CCCGGGCTGCTCAGGGGGGGAGCGGGTGGGCTGGGGATCATGGGGCTGCCTCCTCTGCCA
GCCCCCGGCGAGCCTTGCCCGTTGGCTCAGGAGGAGGTGATTGAGATCAACCGGGCAGAT
CCTCGCCCCAATGGGGATCCGGCAGCAGCTGCGCTTGCCCACGAGGACTGCCCAGCCATT
GACCAGCCTGCCATGTCCCCGGAAGACAAGAGCCCCATCACGCCTGGAAGCCGTGGCCGC
TATAGCCGGGACCGAGCCTGCTTCCTCCTCACCGACTATGCCCCTTCCCCTGATGGCTCC
ATCCGAAAAGCCACTGGTGCTCCCCCACTGCCCCCCCAAGACTGGCGTAAGCCAGGCCCC
CCAAGCTTCTTGCCCGACCTCAACGCCAACGCCGCGGCCTGGATATCCCCCTAG
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:6235
Chromosome Location19
LocusNot Available
References
  1. Rae JL, Shepard AR: Kv3.3 potassium channels in lens epithelium and corneal endothelium. Exp Eye Res. 2000 Mar;70(3):339-48. 10712820
  2. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A, Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA, Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M, Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V, Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S, Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D, Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS, DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA, Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM, Lucas SM: The DNA sequence and biology of human chromosome 19. Nature. 2004 Apr 1;428(6982):529-35. 15057824
  3. Zhao J, Zhu J, Thornhill WB: Spinocerebellar ataxia-13 Kv3.3 potassium channels: arginine-to-histidine mutations affect both functional and protein expression on the cell surface. Biochem J. 2013 Sep 1;454(2):259-65. doi: 10.1042/BJ20130034. 23734863
  4. Gallego-Iradi C, Bickford JS, Khare S, Hall A, Nick JA, Salmasinia D, Wawrowsky K, Bannykh S, Huynh DP, Rincon-Limas DE, Pulst SM, Nick HS, Fernandez-Funez P, Waters MF: KCNC3(R420H), a K(+) channel mutation causative in spinocerebellar ataxia 13 displays aberrant intracellular trafficking. Neurobiol Dis. 2014 Nov;71:270-9. doi: 10.1016/j.nbd.2014.08.020. Epub 2014 Aug 22. 25152487
  5. Waters MF, Minassian NA, Stevanin G, Figueroa KP, Bannister JP, Nolte D, Mock AF, Evidente VG, Fee DB, Muller U, Durr A, Brice A, Papazian DM, Pulst SM: Mutations in voltage-gated potassium channel KCNC3 cause degenerative and developmental central nervous system phenotypes. Nat Genet. 2006 Apr;38(4):447-51. Epub 2006 Feb 26. 16501573
  6. Figueroa KP, Minassian NA, Stevanin G, Waters M, Garibyan V, Forlani S, Strzelczyk A, Burk K, Brice A, Durr A, Papazian DM, Pulst SM: KCNC3: phenotype, mutations, channel biophysics-a study of 260 familial ataxia patients. Hum Mutat. 2010 Feb;31(2):191-6. doi: 10.1002/humu.21165. 19953606
  7. Figueroa KP, Waters MF, Garibyan V, Bird TD, Gomez CM, Ranum LP, Minassian NA, Papazian DM, Pulst SM: Frequency of KCNC3 DNA variants as causes of spinocerebellar ataxia 13 (SCA13). PLoS One. 2011 Mar 29;6(3):e17811. doi: 10.1371/journal.pone.0017811. 21479265
  8. Duarri A, Nibbeling EA, Fokkens MR, Meijer M, Boerrigter M, Verschuuren-Bemelmans CC, Kremer BP, van de Warrenburg BP, Dooijes D, Boddeke E, Sinke RJ, Verbeek DS: Functional analysis helps to define KCNC3 mutational spectrum in Dutch ataxia cases. PLoS One. 2015 Mar 10;10(3):e0116599. doi: 10.1371/journal.pone.0116599. eCollection 2015. 25756792