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Kv11.3

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Target not currently curated in GtoImmuPdb

Target id: 574

Nomenclature: Kv11.3

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 1196 2q24.2 KCNH7 potassium voltage-gated channel subfamily H member 7
Mouse 6 1 1195 2 C1.3 Kcnh7 potassium voltage-gated channel, subfamily H (eag-related), member 7
Rat 6 1 1195 3q21 Kcnh7 potassium voltage-gated channel subfamily H member 7
Previous and Unofficial Names Click here for help
erg3 | HERG3 | eag-related protein 3 | ether-a-go-go-related gene potassium channel 3 | potassium voltage-gated channel, subfamily H (eag-related), member 7 | potassium channel, voltage gated eag related subfamily H, member 7 | potassium voltage-gated channel
Database Links Click here for help
Alphafold Q9NS40 (Hs), Q9ER47 (Mm), O54852 (Rn)
CATH/Gene3D 2.60.120.10
Ensembl Gene ENSG00000184611 (Hs), ENSMUSG00000059742 (Mm), ENSRNOG00000007528 (Rn)
Entrez Gene 90134 (Hs), 170738 (Mm), 170739 (Rn)
Human Protein Atlas ENSG00000184611 (Hs)
KEGG Gene hsa:90134 (Hs), mmu:170738 (Mm), rno:170739 (Rn)
OMIM 608169 (Hs)
Pharos Q9NS40 (Hs)
RefSeq Nucleotide NM_173162 (Hs), NM_033272 (Hs), NM_133207 (Mm), NM_131912 (Rn)
RefSeq Protein NP_775185 (Hs), NP_150375 (Hs), NP_573470 (Mm), NP_571987 (Rn)
UniProtKB Q9NS40 (Hs), Q9ER47 (Mm), O54852 (Rn)
Wikipedia KCNH7 (Hs)
Ion Selectivity and Conductance Click here for help
Species:  Rat
Macroscopic current rectification:  Ik
References:  7,11
Voltage Dependence Click here for help
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  -44.0 20.0 7 Xenopus laevis oocyte Rat
Inactivation  - 10.0 7
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  -42.0 17.0 9,11 CHO cells. Rat
Inactivation  - -
Deactivation  -44.0 7.0 – 22.0 6,9
Voltage Dependence Comments
Kv11.2 and Kv11.3 can form heteromultimers; for voltage dependence data see [11]
Associated subunits (Human)
minK (KCNE1)

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Activators
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
NS1643 Small molecule or natural product Rn - - - 1x10-5 - 1
⤷ Conc range: 1x10-5 M 10 µM NS1643 produced a -15 to -20mV shift in V0.5 of channel activation [1]
Activator Comments
10µM NS1643 produced a -15 to- 20mV shift in V0.5 of channel activation [1].
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
ErgTx-1 Peptide Click here for species-specific activity table Rn Pore blocker 8.4 pKd - - 10
⤷ pKd 8.4 [10]
BeKm-1 Peptide Click here for species-specific activity table Rn Pore blocker 7.9 pKd - - 10
⤷ pKd 7.9 [10]
E4031 Small molecule or natural product Click here for species-specific activity table Ligand has a PDB structure Rn - 6.7 pKd - - 7
⤷ pKd 6.7 [7]
Tissue Distribution Click here for help
Spinal cord and dorsal root ganglia (DRG), largely within GABAergic neurons, widely expressed throughout adult mouse CNS
Species:  Mouse
Technique:  Immunocytochemistry, immunohistochemistry
References:  2-4
Broadly expressed throughout the nervous system (similar to erg1), sympathetic ganglia and low levels in retina
Species:  Rat
Technique:  In situ hybridisation
References:  7,12
All Kv11 transcripts are found in pancreatic islet cells and are under differential spatial and circadian regulation
Species:  Rat
Technique:  Immunohistochemistry
References:  5
Tissue Distribution Comments
Kv11.1, Kv11.2 and Kv11.3 channels are widely expressed throughout the nervous system [7,12]. All, with the exception of Kv11.1b isoform are transiently expressed in the developing spinal cord, largely within GABAergic neurons where they regulate nascent spinal motor rhythms [3]. All are expressed in adult brain in largely overlapping distributions and largely in neuron somata [4].
Physiological Functions Click here for help
Kv11.3 regulates nascent spinal motor rhythms in developing mouse spinal cord
Species:  Mouse
Tissue:  Spinal cord
References:  3
Clinically-Relevant Mutations and Pathophysiology Click here for help
Disease:  Major affective disorder 1; MAFD1
Synonyms: Bipolar affective disorder
Manic depressive-psychosis
OMIM: 125480
Role:  Unknown; R394H mutation shifts voltage dependence of activation to more positive potentials and slows activation kinetics
References:  8
Click column headers to sort
Type Species Amino acid change Nucleotide change Description Reference
Missense Human R394H 8
Biologically Significant Variants Click here for help
Type:  Splice variant
Species:  Human
Description:  Kv11.3 isoform 2
Amino acids:  732
Nucleotide accession:  NM_173162
Protein accession:  NP_775185
Type:  Splice variant
Species:  Human
Description:  Kv11.3 isoform 1
Amino acids:  1196
Nucleotide accession:  NM_033272
Protein accession:  NP_150375

References

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1. Bilet A, Bauer CK. (2012) Effects of the small molecule HERG activator NS1643 on Kv11.3 channels. PLoS ONE, 7 (11): e50886. [PMID:23226420]

2. Furlan F, Guasti L, Avossa D, Becchetti A, Cilia E, Ballerini L, Arcangeli A. (2005) Interneurons transiently express the ERG K+ channels during development of mouse spinal networks in vitro. Neuroscience, 135 (4): 1179-92. [PMID:16165280]

3. Furlan F, Taccola G, Grandolfo M, Guasti L, Arcangeli A, Nistri A, Ballerini L. (2007) ERG conductance expression modulates the excitability of ventral horn GABAergic interneurons that control rhythmic oscillations in the developing mouse spinal cord. J Neurosci, 27 (4): 919-28. [PMID:17251434]

4. Guasti L, Cilia E, Crociani O, Hofmann G, Polvani S, Becchetti A, Wanke E, Tempia F, Arcangeli A. (2005) Expression pattern of the ether-a-go-go-related (ERG) family proteins in the adult mouse central nervous system: evidence for coassembly of different subunits. J Comp Neurol, 491 (2): 157-74. [PMID:16127690]

5. Mühlbauer E, Bazwinsky I, Wolgast S, Klemenz A, Peschke E. (2007) Circadian changes of ether-a-go-go-related-gene (Erg) potassium channel transcripts in the rat pancreas and beta-cell. Cell Mol Life Sci, 64 (6): 768-80. [PMID:17322986]

6. Schledermann W, Wulfsen I, Schwarz JR, Bauer CK. (2001) Modulation of rat erg1, erg2, erg3 and HERG K+ currents by thyrotropin-releasing hormone in anterior pituitary cells via the native signal cascade. J Physiol (Lond.), 532 (Pt 1): 143-63. [PMID:11283231]

7. Shi W, Wymore RS, Wang HS, Pan Z, Cohen IS, McKinnon D, Dixon JE. (1997) Identification of two nervous system-specific members of the erg potassium channel gene family. J Neurosci, 17 (24): 9423-32. [PMID:9390998]

8. Strauss KA, Markx S, Georgi B, Paul SM, Jinks RN, Hoshi T, McDonald A, First MB, Liu W, Benkert AR et al.. (2014) A population-based study of KCNH7 p.Arg394His and bipolar spectrum disorder. Hum Mol Genet, 23 (23): 6395-406. [PMID:24986916]

9. Sturm P, Wimmers S, Schwarz JR, Bauer CK. (2005) Extracellular potassium effects are conserved within the rat erg K+ channel family. J Physiol (Lond.), 564 (Pt 2): 329-45. [PMID:15705650]

10. Wanke E, Restano-Cassulini R. (2007) Toxins interacting with ether-à-go-go-related gene voltage-dependent potassium channels. Toxicon, 49 (2): 239-48. [PMID:17097705]

11. Wimmers S, Bauer CK, Schwarz JR. (2002) Biophysical properties of heteromultimeric erg K+ channels. Pflugers Arch, 445 (3): 423-30. [PMID:12466946]

12. Wymore RS, Gintant GA, Wymore RT, Dixon JE, McKinnon D, Cohen IS. (1997) Tissue and species distribution of mRNA for the IKr-like K+ channel, erg. Circ Res, 80 (2): 261-8. [PMID:9012748]

Contributors

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