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NamePotassium voltage-gated channel subfamily C member 1
Synonyms
  • NGK2
  • Voltage-gated potassium channel subunit Kv3.1
  • Voltage-gated potassium channel subunit Kv4
Gene NameKCNC1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0008574|Potassium voltage-gated channel subfamily C member 1
MGQGDESERIVINVGGTRHQTYRSTLRTLPGTRLAWLAEPDAHSHFDYDPRADEFFFDRH
PGVFAHILNYYRTGKLHCPADVCGPLYEEELAFWGIDETDVEPCCWMTYRQHRDAEEALD
SFGGAPLDNSADDADADGPGDSGDGEDELEMTKRLALSDSPDGRPGGFWRRWQPRIWALF
EDPYSSRYARYVAFASLFFILVSITTFCLETHERFNPIVNKTEIENVRNGTQVRYYREAE
TEAFLTYIEGVCVVWFTFEFLMRVIFCPNKVEFIKNSLNIIDFVAILPFYLEVGLSGLSS
KAAKDVLGFLRVVRFVRILRIFKLTRHFVGLRVLGHTLRASTNEFLLLIIFLALGVLIFA
TMIYYAERIGAQPNDPSASEHTHFKNIPIGFWWAVVTMTTLGYGDMYPQTWSGMLVGALC
ALAGVLTIAMPVPVIVNNFGMYYSLAMAKQKLPKKKKKHIPRPPQLGSPNYCKSVVNSPH
HSTQSDTCPLAQEEILEINRAGRKPLRGMSI
Number of residues511
Molecular Weight57941.87
Theoretical pINot Available
GO Classification
Functions
  • voltage-gated potassium channel activity
  • delayed rectifier potassium channel activity
Processes
  • response to toxic substance
  • cerebellum development
  • cellular response to drug
  • response to amine
  • protein homooligomerization
  • positive regulation of potassium ion transmembrane transport
  • potassium ion transport
  • response to fibroblast growth factor
  • synaptic transmission
  • response to auditory stimulus
  • response to potassium ion
  • globus pallidus development
  • protein tetramerization
  • response to light intensity
  • response to nerve growth factor
  • potassium ion transmembrane transport
Components
  • axolemma
  • dendrite membrane
  • neuronal cell body membrane
  • plasma membrane
  • voltage-gated potassium channel complex
  • cell surface
  • integral component of membrane
  • dendrite
General FunctionVoltage-gated potassium channel activity
Specific FunctionMediates 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. Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNC2, and possibly other family members as well. Contributes to fire sustained trains of very brief action potentials at high frequency in pallidal neurons.
Pfam Domain Function
Transmembrane Regions191-209 248-267 277-295 309-331 345-366 415-436
GenBank Protein IDNot Available
UniProtKB IDP48547
UniProtKB Entry NameKCNC1_HUMAN
Cellular LocationCell membrane
Gene sequence
>lcl|BSEQ0021874|Potassium voltage-gated channel subfamily C member 1 (KCNC1)
ATGGGCCAAGGGGACGAGAGCGAGCGCATCGTGATCAACGTGGGCGGCACGCGCCACCAG
ACGTACCGCTCGACCCTGCGCACGCTGCCCGGCACGCGGCTCGCCTGGCTGGCGGAGCCC
GACGCCCACAGCCACTTCGACTATGACCCGCGTGCTGACGAGTTCTTCTTCGACCGCCAC
CCCGGCGTCTTCGCGCACATCCTGAACTACTACCGCACGGGCAAGCTGCACTGCCCAGCC
GACGTGTGCGGGCCGCTCTACGAGGAGGAGCTGGCCTTCTGGGGCATCGACGAGACCGAC
GTGGAGCCCTGCTGCTGGATGACGTACCGCCAGCACCGCGACGCCGAGGAGGCTCTGGAC
AGCTTCGGCGGCGCTCCTCTGGACAACAGCGCCGACGACGCGGACGCCGACGGCCCTGGC
GACTCGGGCGACGGCGAGGACGAGCTGGAGATGACCAAGCGCCTGGCGCTCAGTGACTCC
CCGGATGGCCGGCCTGGCGGCTTTTGGCGCCGCTGGCAGCCGCGCATCTGGGCGCTCTTC
GAGGACCCGTACTCGTCCCGCTACGCGCGGTATGTGGCCTTCGCTTCCCTCTTCTTCATC
CTGGTCTCCATCACCACCTTCTGCCTGGAGACCCACGAGCGCTTCAACCCCATCGTGAAC
AAGACGGAGATCGAGAACGTTCGCAATGGCACGCAAGTGCGCTACTACCGGGAGGCCGAG
ACGGAGGCCTTCCTTACCTACATCGAGGGCGTCTGTGTGGTCTGGTTCACCTTCGAGTTC
CTCATGCGTGTCATCTTCTGCCCCAACAAGGTAGAGTTCATCAAGAACTCGCTCAACATC
ATTGACTTTGTGGCCATCCTGCCCTTCTACCTGGAGGTGGGGCTGAGCGGCCTGTCCTCC
AAGGCAGCCAAGGACGTGCTGGGCTTCCTGCGCGTCGTCCGCTTCGTGCGCATCTTGCGC
ATCTTTAAGCTGACCCGCCACTTTGTGGGCCTGCGGGTCCTGGGCCACACGCTCCGAGCC
AGCACCAACGAGTTCCTGCTGCTCATCATCTTCCTGGCCTTGGGCGTGCTGATCTTCGCC
ACCATGATCTACTACGCCGAGAGGATAGGGGCACAGCCCAATGACCCCAGCGCCAGTGAG
CACACGCACTTTAAGAACATCCCCATCGGCTTCTGGTGGGCCGTGGTCACCATGACGACC
CTGGGCTATGGAGACATGTACCCGCAGACGTGGTCCGGCATGCTGGTGGGGGCTCTGTGT
GCGCTGGCGGGCGTGCTCACCATCGCCATGCCCGTGCCCGTCATCGTGAACAATTTCGGG
ATGTATTACTCCTTAGCCATGGCTAAGCAGAAACTACCAAAGAAAAAAAAGAAGCATATT
CCGCGGCCACCGCAGCTGGGATCTCCCAATTATTGTAAATCTGTCGTAAACTCTCCACAC
CACAGTACTCAGAGTGACACATGTCCGCTGGCCCAGGAAGAAATTTTAGAAATTAACAGA
GCAGATTCCAAACTGAATGGGGAGGTGGCGAAGGCCGCGCTGGCGAACGAAGACTGCCCC
CACATAGACCAGGCCCTCACTCCCGATGAGGGCCTGCCCTTTACGCGCTCGGGCACCCGC
GAGAGATACGGACCCTGCTTCCTCTTATCAACCGGGGAGTACGCGTGCCCACCTGGTGGA
GGAATGAGAAAGGATCTTTGCAAAGAAAGCCCTGTCATTGCTAAGTATATGCCGACAGAG
GCTGTGAGAGTGACTTGA
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:6233
Chromosome Location11
LocusNot Available
References
  1. Ried T, Rudy B, Vega-Saenz de Miera E, Lau D, Ward DC, Sen K: Localization of a highly conserved human potassium channel gene (NGK2-KV4; KCNC1) to chromosome 11p15. Genomics. 1993 Feb;15(2):405-11. 8449507
  2. Taylor TD, Noguchi H, Totoki Y, Toyoda A, Kuroki Y, Dewar K, Lloyd C, Itoh T, Takeda T, Kim DW, She X, Barlow KF, Bloom T, Bruford E, Chang JL, Cuomo CA, Eichler E, FitzGerald MG, Jaffe DB, LaButti K, Nicol R, Park HS, Seaman C, Sougnez C, Yang X, Zimmer AR, Zody MC, Birren BW, Nusbaum C, Fujiyama A, Hattori M, Rogers J, Lander ES, Sakaki Y: Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature. 2006 Mar 23;440(7083):497-500. 16554811
  3. Grissmer S, Ghanshani S, Dethlefs B, McPherson JD, Wasmuth JJ, Gutman GA, Cahalan MD, Chandy KG: The Shaw-related potassium channel gene, Kv3.1, on human chromosome 11, encodes the type l K+ channel in T cells. J Biol Chem. 1992 Oct 15;267(29):20971-9. 1400413
  4. 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
  5. Muona M, Berkovic SF, Dibbens LM, Oliver KL, Maljevic S, Bayly MA, Joensuu T, Canafoglia L, Franceschetti S, Michelucci R, Markkinen S, Heron SE, Hildebrand MS, Andermann E, Andermann F, Gambardella A, Tinuper P, Licchetta L, Scheffer IE, Criscuolo C, Filla A, Ferlazzo E, Ahmad J, Ahmad A, Baykan B, Said E, Topcu M, Riguzzi P, King MD, Ozkara C, Andrade DM, Engelsen BA, Crespel A, Lindenau M, Lohmann E, Saletti V, Massano J, Privitera M, Espay AJ, Kauffmann B, Duchowny M, Moller RS, Straussberg R, Afawi Z, Ben-Zeev B, Samocha KE, Daly MJ, Petrou S, Lerche H, Palotie A, Lehesjoki AE: A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy. Nat Genet. 2015 Jan;47(1):39-46. doi: 10.1038/ng.3144. Epub 2014 Nov 17. 25401298