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Kv3.3

Family: Voltage-gated potassium channels

Contents:
Gene and Protein Information
Previous and Unofficial Names
Database Links
Ion Selectivity and Conductance
Voltage Dependence
Pore Blockers
Tissue Distribution
Functional Assays
Physiological Functions
Phenotypes, Alleles and Disease Models
Clinically-Relevant Mutations and Pathophysiology
General Comments
References
Gene and Protein Information
Species TM P Loops AA Chromosomal Location Gene Symbol Gene Name Reference
Human 6 1 757 19q13.3-13.4 KCNC3 potassium voltage-gated channel, Shaw-related subfamily, member 3 7,9,14
Mouse 6 1 769 7 B4 Kcnc3 potassium voltage gated channel, Shaw-related subfamily, member 3 7,9,14
Rat 6 1 769 1q22 Kcnc3 potassium voltage gated channel, Shaw-related subfamily, member 3 14
Previous and Unofficial Names
hkv3.3
mkv3.3
kv3.3b
RKShIIID
SCA13
Kv3.3
spinocerebellar ataxia 13
KShIIID
potassium voltage-gated channel subfamily C member 3
voltage-gated potassium channel subunit Kv3.3
Kcr2-3
Database Links
Ensembl ENSG00000131398 (Hs), ENSMUSG00000062785 (Mm), ENSRNOG00000019959 (Rn)
Entrez Gene 3748 (Hs), 16504 (Mm), 117101 (Rn)
GeneCards KCNC3 (Hs)
GenitoUrinary Development Molecular Anatomy Project Kcnc3 (Mm)
HomoloGene 3650 (Hs)
Human Protein Reference Database 08886 (Hs)
InterPro Q14003 (Hs), Q63959 (Mm), Q01956 (Rn)
KEGG Gene hsa:3748 (Hs), mmu:16504 (Mm), rno:117101 (Rn)
OMIM 176264 (Hs)
Orphanet Gene ORPHA159737 (Hs)
PharmGKB Gene PA30027 (Hs)
PhosphoSitePlus Q14003 (Hs), Q63959 (Mm)
Protein Ontology (PRO) PRO:000009254 (Hs)
RefSeq Nucleotide NM_004977 (Hs), NM_008422 (Mm), NM_053997 (Rn)
RefSeq Protein NP_004968 (Hs), NP_032448 (Mm), NP_446449 (Rn)
TreeFam ENSG00000131398 (Hs), ENSMUSG00000062785 (Mm), ENSRNOG00000019959 (Rn)
UniGene Hs. 467146 (Hs)
UniProt Q14003 (Hs), Q63959 (Mm), Q01956 (Rn)
Wikipedia Kv3.3
Potassium channels
Search for 3D structures on the PDB
Search by keyword: Voltage-gated potassium channels Kv3.3 Search by accession number: Q01956, Q63959, Q14003
Ion Selectivity and Conductance
Species:  Rat
Macroscopic current rectification:  A-Type current
References:  14
Ion Selectivity and Conductance Comments
K+ ion selectivity; details not established
Voltage Dependence
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  7.0 - 14 Xenopus laevis oocyte Rat
Inactivation  - 200.0 14
Voltage Dependence Comments
Also carried out in CHO and HEK293 cells transfected with the teleost homologue of Kv3.3 known as AptKv3.3 [6]
Pore Blockers
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Affinity Units Concentration range (M) Holding voltage (mV) Reference
tetraethylammonium Rn 3.8 pIC50 - - 14
4-aminopyridine Rn 2.9 pIC50 - - 14
Tissue Distribution
Myocardium
Species:  Human
Technique:  RT-PCR
References:  11
Brain (auditory nuclei, cerebellar Purkinje cells)
Species:  Mouse
Technique:  Immunohistochemistry
References:  4,8,13
Medulla Oblongata and spinal cord
Species:  Rat
Technique:  Immunohistochemistry
References:  3
Brain (brainstem and cerebellum > forebrain), axons, terminals, somas and distal dendrites, Purkinje cells, central nervous system motorneurons, auditory brainstem, mesenteric artery.
Species:  Rat
Technique:  Immunohistochemistry
References:  1,15
Tissue Distribution Comments
Also detected in rabbit lens and corneal epithelium [12]
Functional Assays
Voltage clamp
Species:  Rat
Tissue:  Xenopus laevis oocytes
Response measured:  Voltage-gated A-type potassium channel
References:  14
Physiological Functions
Controls the rate of spontaneous action potential firing of Purkinje neurons and regulates the dynamics of synaptic transmission.
Species:  Mouse
Tissue:  Cerebellum, Purkinje cells.
References:  2
Involved in motor control, such as cortex, basal ganglia and cerrebellum.
Species:  Mouse
Tissue:  Brain
References:  10
Physiological Consequences of Altering Gene Expression
Not Determined
Phenotypes, Alleles and Disease Models Mouse data from MGI

Click here to show/hide data

Allele Composition & genetic background Accession Phenotype Id Phenotype Reference
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001393 ataxia PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001265 decreased body size PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0005534 decreased body temperature PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001262 decreased body weight PMID: 11517255 
Kcnc1+|Kcnc1tm1Joho|Kcnc3+|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1+,Kcnc3tm1Echa/Kcnc3+
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001262 decreased body weight PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0009751 enhanced behavioral response to alcohol PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001399 hyperactivity PMID: 11517255 
Kcnc1+|Kcnc1tm1Joho|Kcnc3+|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1+,Kcnc3tm1Echa/Kcnc3+
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001399 hyperactivity PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001525 impaired balance PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001405 impaired coordination PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001415 increased exploration in new environment PMID: 11517255 
Kcnc1+|Kcnc1tm1Joho|Kcnc3+|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1+,Kcnc3tm1Echa/Kcnc3+
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001415 increased exploration in new environment PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0005424 jerky movement PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0008569 lethality at weaning PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0000243 myoclonus PMID: 11517255 
Kcnc3tm1Echa Kcnc3tm1Echa/Kcnc3tm1Echa
Not Specified		 MGI:96669  MP:0002169 no abnormal phenotype detected PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0008489 postnatal slow weight gain PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0000745 tremors PMID: 11517255 
Kcnc1tm1Joho|Kcnc3tm1Echa Kcnc1tm1Joho/Kcnc1tm1Joho,Kcnc3tm1Echa/Kcnc3tm1Echa
involves: 129/Sv * C57BL/6		 MGI:96667  MGI:96669  MP:0001263 weight loss PMID: 11517255 
Clinically-Relevant Mutations and Pathophysiology
Disease:  Spinocerebellar ataxia 13; SCA13
OMIM:  605259
Orphanet:  98768
References: 
Mutations not determined
Gene Expression and Pathophysiology
Not Determined
Biologically Significant Variants
Not Determined
General Comments
Mice lacking Kv3.3 and Kv3.1 show alcohol hypersensitivity, increased locomotion and myoclonus [5]
It is a member of the mammalian Shaw-related family.

REFERENCES

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1. Adelman, JP; Bond, CT; Pessia, M; Maylie, J. (1995) Episodic ataxia results from voltage-dependent potassium channels with altered functions. Neuron, 15 (6): 1449-54. [PMID:8845167]

2. Akemann, W; Knöpfel, T. (2006) Interaction of Kv3 potassium channels and resurgent sodium current influences the rate of spontaneous firing of Purkinje neurons. J. Neurosci., 26 (17): 4602-12. [PMID:16641240]

3. Brooke, RE; Atkinson, L; Edwards, I; Parson, SH; Deuchars, J. (2006) Immunohistochemical localisation of the voltage gated potassium ion channel subunit Kv3.3 in the rat medulla oblongata and thoracic spinal cord. Brain Res., 1070 (1): 101-15. [PMID:16403474]

4. Chang, SY; Zagha, E; Kwon, ES; Ozaita, A; Bobik, M; Martone, ME; Ellisman, MH; Heintz, N; Rudy, B. (2007) Distribution of Kv3.3 potassium channel subunits in distinct neuronal populations of mouse brain. J. Comp. Neurol., 502 (6): 953-72. [PMID:17444489]

5. Espinosa, F; McMahon, A; Chan, E; Wang, S; Ho, CS; Heintz, N; Joho, RH. (2001) Alcohol hypersensitivity, increased locomotion, and spontaneous myoclonus in mice lacking the potassium channels Kv3.1 and Kv3.3. J. Neurosci., 21 (17): 6657-65. [PMID:11517255]

6. Fernandez, FR; Morales, E; Rashid, AJ; Dunn, RJ; Turner, RW. (2003) Inactivation of Kv3.3 potassium channels in heterologous expression systems. J. Biol. Chem., 278 (42): 40890-8. [PMID:12923191]

7. Ghanshani, S; Pak, M; McPherson, JD; Strong, M; Dethlefs, B; Wasmuth, JJ; Salkoff, L; Gutman, GA; Chandy, KG. (1992) Genomic organization, nucleotide sequence, and cellular distribution of a Shaw-related potassium channel gene, Kv3.3, and mapping of Kv3.3 and Kv3.4 to human chromosomes 19 and 1. Genomics, 12 (2): 190-6. [PMID:1740329]

8. Grigg, JJ; Brew, HM; Tempel, BL. (2000) Differential expression of voltage-gated potassium channel genes in auditory nuclei of the mouse brainstem. Hear. Res., 140 (1-2): 77-90. [PMID:10675636]

9. Haas, M; Ward, DC; Lee, J; Roses, AD; Clarke, V; D'Eustachio, P; Lau, D; Vega-Saenz de Miera, E; Rudy, B. (1993) Localization of Shaw-related K+ channel genes on mouse and human chromosomes. Mamm. Genome, 4 (12): 711-5. [PMID:8111118]

10. Joho, RH; Street, C; Matsushita, S; Knöpfel, T. (2006) Behavioral motor dysfunction in Kv3-type potassium channel-deficient mice. Genes Brain Behav., 5 (6): 472-82. [PMID:16923152]

11. Ordög, B; Brutyó, E; Puskás, LG; Papp, JG; Varró, A; Szabad, J; Boldogkoi, Z. (2006) Gene expression profiling of human cardiac potassium and sodium channels. Int. J. Cardiol., 111 (3): 386-93. [PMID:16257073]

12. Rae, JL; Shepard, AR. (2000) Kv3.3 potassium channels in lens epithelium and corneal endothelium. Exp. Eye Res., 70 (3): 339-48. [PMID:10712820]

13. Sacco, T; De Luca, A; Tempia, F. (2006) Properties and expression of Kv3 channels in cerebellar Purkinje cells. Mol. Cell. Neurosci., 33 (2): 170-9. [PMID:16949837]

14. Vega-Saenz de Miera, E; Moreno, H; Fruhling, D; Kentros, C; Rudy, B. (1992) Cloning of ShIII (Shaw-like) cDNAs encoding a novel high-voltage-activating, TEA-sensitive, type-A K+ channel. Proc. Biol. Sci., 248 (1321): 9-18. [PMID:1381835]

15. Xu, C; Lu, Y; Tang, G; Wang, R. (1999) Expression of voltage-dependent K(+) channel genes in mesenteric artery smooth muscle cells. Am. J. Physiol., 277 (5 Pt 1): G1055-63. [PMID:10564112]

To cite this database page, please use the following:

K. George Chandy, Stephan Grissmer, George A. Gutman, Michel Lazdunski, David Mckinnon, Luis A. Pardo, Gail A. Robertson, Bernardo Rudy, Michael C. Sanguinetti, Walter Stühmer, Xiaoliang Wang.
Voltage-gated potassium channels: Kv3.3. Last modified on 23/01/2012. Accessed on 25/05/2013. IUPHAR database (IUPHAR-DB), http://www.iuphar-db.org/DATABASE/ObjectDisplayForward?objectId=550.


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