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Kv3.4

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Target not currently curated in GtoImmuPdb

Target id: 551

Nomenclature: Kv3.4

Family: Voltage-gated potassium channels (Kv)

Contents:

Gene and Protein Information Click here for help
Species TM P Loops AA Chromosomal Location Gene Symbol Gene Name Reference
Human 6 1 635 1p13.3 KCNC4 potassium voltage-gated channel subfamily C member 4 10-11
Mouse 6 1 628 3 46.83 cM Kcnc4 potassium voltage gated channel, Shaw-related subfamily, member 4 11
Rat 6 1 625 2q34 Kcnc4 potassium voltage-gated channel subfamily C member 4 11
Previous and Unofficial Names Click here for help
Raw3 | Kcnc4 | potassium voltage-gated channel subfamily C member 4 | Kcr2-4 | potassium voltage gated channel, Shaw-related subfamily, member 4 | potassium voltage-gated channel, Shaw-related subfamily, member 4 | potassium channel, voltage gated Shaw related subfamily C, member 4 | potassium channel, voltage gated Shaw-related subfamily C, member 4 | potassium voltage gated channel
Database Links Click here for help
Alphafold Q03721 (Hs), Q8R1C0 (Mm), Q63734 (Rn)
Ensembl Gene ENSG00000116396 (Hs), ENSMUSG00000027895 (Mm), ENSRNOG00000060988 (Rn)
Entrez Gene 3749 (Hs), 99738 (Mm), 684516 (Rn)
Human Protein Atlas ENSG00000116396 (Hs)
KEGG Gene hsa:3749 (Hs), mmu:99738 (Mm), rno:684516 (Rn)
OMIM 176265 (Hs)
Pharos Q03721 (Hs)
RefSeq Nucleotide NM_004978 (Hs), NM_145922 (Mm), NM_001122776 (Rn)
RefSeq Protein NP_004969 (Hs), NP_666034 (Mm), NP_001116248 (Rn)
UniProtKB Q03721 (Hs), Q8R1C0 (Mm), Q63734 (Rn)
Wikipedia KCNC4 (Hs)
Associated Proteins Click here for help
Heteromeric Pore-forming Subunits
Name References
Not determined
Auxiliary Subunits
Name References
MinK-related Peptide 2 1
Other Associated Proteins
Name References
Not determined
Functional Characteristics Click here for help
KA
Ion Selectivity and Conductance Click here for help
Species:  Rat
Rank order:  K+ [14.0 pS]
References:  8,11
Species:  Rat
Macroscopic current rectification:  Rapidly activating and inactivating A-Type current
References:  11
Voltage Dependence Click here for help
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  14.0 - 11 Xenopus laevis oocyte Rat
Inactivation  - -
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  -10.0 - 10 Xenopus laevis oocyte Human
Inactivation  - -
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  3.4 - 8 Xenopus laevis oocyte Rat
Inactivation  53.0 16.0 8,10-11
Comments  Fast inactivating
Associated subunits (Human)
MiRP2 is an associated subunit for Kv3.4

Download all structure-activity data for this target as a CSV file go icon to follow link

Gating inhibitors Click here for help
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Concentration range (M) Holding voltage (mV) Reference
thiopental Small molecule or natural product Approved drug Rn - 4.0 pIC50 - - 12
⤷ pIC50 4.0 [12]
Channel Blockers
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Concentration range (M) Holding voltage (mV) Reference
sea anemone toxin BDS-I Peptide Rn - 7.3 pIC50 - - 3
⤷ pIC50 7.3 (IC50 4.7x10-8 M) [3]
DABCO-C16 Small molecule or natural product Click here for species-specific activity table Rn - 6.2 pIC50 - - 4
⤷ pIC50 6.2 [4]
tetraethylammonium Small molecule or natural product Click here for species-specific activity table Ligand has a PDB structure Rn - 3.5 pIC50 - - 8,11
⤷ pIC50 3.5 (IC50 3x10-4 M) [8,11]
DABCO Small molecule or natural product Rn - 3.2 pIC50 - - 4
⤷ pIC50 3.2 [4]
Channel Blocker Comments
The specificity of BDS-I for Kv3.4 has been questioned.
Tissue Distribution Click here for help
Acinar cells
Species:  Mouse
Technique:  RT-PCR
References:  5-6
Parathyroid, prostate, brain
Species:  Rat
Technique:  RT-PCR
References:  9
Brainstem, hippocampal granule cells, skeletal muscle
Species:  Rat
Technique:  Northern Blot
References:  13
Functional Assays Click here for help
Whole cell voltage clamp
Species:  Rat
Tissue:  Xenopus laevis oocytes
Response measured:  Voltage-gated potassium channel, A-type, fast inactivating
References:  11
Physiological Functions Click here for help
In combination with MirP2, Kv3.4 forms low-voltage activating potassium channels that regulate skeletal muscle resting potential.
Species:  Rat
Tissue:  Rat skeletal muscle tissue
References:  1
Clinically-Relevant Mutations and Pathophysiology Click here for help
Disease:  Periodic paralysis
References:  1-2
Click column headers to sort
Type Species Amino acid change Nucleotide change Description Reference
Missense Human R83H 1-2
Gene Expression and Pathophysiology Click here for help
Increase in Kv3.4 and MirP2 transcripts and proteins induced after treatment with Aβ1-42
Tissue or cell type:  Hippocampal neurons and PC-12 cells.
Pathophysiology:  Neurodegeneration
Species:  Rat
Technique:  PC-12 cells
References:  7
General Comments
Kv3.4 belongs to the mammalian Shaw-related family.

References

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1. Abbott GW, Butler MH, Bendahhou S, Dalakas MC, Ptacek LJ, Goldstein SA. (2001) MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis. Cell, 104 (2): 217-31. [PMID:11207363]

2. Abbott GW, Butler MH, Goldstein SA. (2006) Phosphorylation and protonation of neighboring MiRP2 sites: function and pathophysiology of MiRP2-Kv3.4 potassium channels in periodic paralysis. FASEB J, 20 (2): 293-301. [PMID:16449802]

3. Diochot S, Schweitz H, Béress L, Lazdunski M. (1998) Sea anemone peptides with a specific blocking activity against the fast inactivating potassium channel Kv3.4. J Biol Chem, 273 (12): 6744-9. [PMID:9506974]

4. Gordon E, Cohen JL, Engel R, Abbott GW. (2006) 1,4-Diazabicyclo[2.2.2]octane derivatives: a novel class of voltage-gated potassium channel blockers. Mol Pharmacol, 69 (3): 718-26. [PMID:16317109]

5. Göpel SO, Kanno T, Barg S, Rorsman P. (2000) Patch-clamp characterisation of somatostatin-secreting -cells in intact mouse pancreatic islets. J Physiol (Lond.), 528 (Pt 3): 497-507. [PMID:11060127]

6. Kalman K, Nguyen A, Tseng-Crank J, Dukes ID, Chandy G, Hustad CM, Copeland NG, Jenkins NA, Mohrenweiser H, Brandriff B et al.. (1998) Genomic organization, chromosomal localization, tissue distribution, and biophysical characterization of a novel mammalian Shaker-related voltage-gated potassium channel, Kv1.7. J Biol Chem, 273 (10): 5851-7. [PMID:9488722]

7. Pannaccione A, Boscia F, Scorziello A, Adornetto A, Castaldo P, Sirabella R, Taglialatela M, Di Renzo GF, Annunziato L. (2007) Up-regulation and increased activity of KV3.4 channels and their accessory subunit MinK-related peptide 2 induced by amyloid peptide are involved in apoptotic neuronal death. Mol Pharmacol, 72 (3): 665-73. [PMID:17495071]

8. Rettig J, Wunder F, Stocker M, Lichtinghagen R, Mastiaux F, Beckh S, Kues W, Pedarzani P, Schröter KH, Ruppersberg JP. (1992) Characterization of a Shaw-related potassium channel family in rat brain. EMBO J, 11 (7): 2473-86. [PMID:1378392]

9. Riazanski V, Becker A, Chen J, Sochivko D, Lie A, Wiestler OD, Elger CE, Beck H. (2001) Functional and molecular analysis of transient voltage-dependent K+ currents in rat hippocampal granule cells. J Physiol (Lond.), 537 (Pt 2): 391-406. [PMID:11731573]

10. Rudy B, Sen K, Vega-Saenz de Miera E, Lau D, Ried T, Ward DC. (1991) Cloning of a human cDNA expressing a high voltage-activating, TEA-sensitive, type-A K+ channel which maps to chromosome 1 band p21. J Neurosci Res, 29 (3): 401-12. [PMID:1920536]

11. Schröter KH, Ruppersberg JP, Wunder F, Rettig J, Stocker M, Pongs O. (1991) Cloning and functional expression of a TEA-sensitive A-type potassium channel from rat brain. FEBS Lett, 278 (2): 211-6. [PMID:1840526]

12. Suzuki H, Momoi N, Ono T, Maeda S, Shikama Y, Matsuoka I, Suzuki H, Kimura J. (2005) Inhibitory effect of thiopental on ultra-rapid delayed rectifier K+ current in H9c2 cells. J Pharmacol Sci, 99 (2): 177-84. [PMID:16217144]

13. Weiser M, Vega-Saenz de Miera E, Kentros C, Moreno H, Franzen L, Hillman D, Baker H, Rudy B. (1994) Differential expression of Shaw-related K+ channels in the rat central nervous system. J Neurosci, 14 (3 Pt 1): 949-72. [PMID:8120636]

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