image of a green circle Annotated and expert reviewed. Please contact us if you can help with updates. 

Kv7.2

Family: Voltage-gated potassium channels

Contents:
Gene and Protein Information
Previous and Unofficial Names
Database Links
Associated Proteins
Ion Selectivity and Conductance
Voltage Dependence
Activators
Pore Blockers
Tissue Distribution
Physiological Functions
Phenotypes, Alleles and Disease Models
Clinically-Relevant Mutations and Pathophysiology
Biologically Significant Variants
General Comments
References
Gene and Protein Information
Species TM P Loops AA Chromosomal Location Gene Symbol Gene Name Reference
Human 6 1 872 20q13.3 KCNQ2 potassium voltage-gated channel, KQT-like subfamily, member 2 22
Mouse 6 1 870 2 H3-4 Kcnq2 potassium voltage-gated channel, subfamily Q, member 2 16
Rat 6 1 852 3q43 Kcnq2 potassium voltage-gated channel, KQT-like subfamily, member 2 11
Previous and Unofficial Names
KQT2
Kv7.2
KCNQ2
EBN
EBN1
ENB1
BFNC
KCNA11
HNSPC
KQT-like 2
potassium channel subunit alpha KvLQT2
potassium voltage-gated channel subfamily KQT member 2
potassium voltage-gated channel, KQT-like subfamily, member 2
potassium voltage-gated channel, subfamily Q, member 2
voltage-gated potassium channel subunit Kv7.2
Nmf134
Database Links
ChEMBL Target
DrugBank Target
Ensembl
Entrez Gene
GeneCards
GenitoUrinary Development Molecular Anatomy Project
HomoloGene
Human Protein Reference Database
InterPro
KEGG Gene
OMIM
Orphanet Gene
PharmGKB Gene
PhosphoSitePlus
Protein Ontology (PRO)
RefSeq Nucleotide
RefSeq Protein
TreeFam
UniGene Hs.
UniProt
Wikipedia
Search for 3D structures on the PDB
Search by keyword: Voltage-gated potassium channels Kv7.2
Associated Proteins
Heteromeric Pore-forming Subunits
Name References
KCNQ3 10,20,27
Auxiliary Subunits
Name References
Not determined
Other Associated Proteins
Name References
Not determined
Ion Selectivity and Conductance
Species:  Human
Single channel conductance (pS):  6.2
References:  13,24
Ion Selectivity and Conductance Comments
  • human KCNQ2 ion selectivity ranking: K+>Rb+>Cs+>Na+ [3]
  • human KCNQ2/3 permeation sequence : Tl+>K+>Rb+>NH4+>Cs+>Na+ [18]
  • human KCNQ2/3 conduction sequence : K+>Tl+>NH4+~Rb+>Cs+ [18]
Voltage Dependence
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  -37.0 – -40.0 132.0 3,14,19 Xenopus laevis oocyte Human
Inactivation  - -
  V0.5 (mV)  τ (msec)  Reference  Cell type  Species 
Activation  -11.5 – -40.0 129.0 8,24-25 CHO cells Human
Inactivation  - -
Associated subunits (Human)
Activators
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
zinc pyrithione Hs 5.8 pEC50 - - 30
retigabine Hs 5.6 pEC50 - - 25
N-(S)-MPEPAamide Hs 5.5 pEC50 - - 2,29
BMS204352 Hs 5.0 pEC50 - - 21
flupirtine Hs 5.0 pEC50 - - 15
PIP2 Hs 3.7 pEC50 - - 12
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
linopirdine Hs 5.32 - - - 27
XE991 Hs 6.15 pIC50 - - 27
L735821 Hs 5.8 pIC50 - - 26
tetraethylammonium Hs 3.9 – 3.5 (median: 3.8) pIC50 - - 9,27-28
Tissue Distribution
cortex, hippocampus
Species:  Human
Technique:  Immunohistochemistry
References:  5
Predominantly expressed in brain
Species:  Human
Technique:  Northern Blot
References:  20,31
basal ganglia, septum, thalamus, hippocampus (wide brain distribution)
Species:  Mouse
Technique:  Immunohistochemistry
References:  6
sympathetic ganglia, high expression levels in the cerebellum, cortex and hippocampus
Species:  Rat
Technique:  Northern Blot
References:  27
broad distribution in brain
Species:  Rat
Technique:  In situ hybridisation
References:  20
Physiological Functions
Probably important in the regulation of neuronal excitability. Associates with KCNQ3 to form a potassium channel with essentially identical properties to the channel underlying the native M-current
Species:  Human
Tissue:  neurons
References:  5,20,27
Immunoreactivity in some terminal fields for KCNQ2, but not KCNQ3, suggests a presynaptic role in the regulation of action potential propagation and neurotransmitter release
Species:  Human
Tissue:  Brain and CNS
References:  5
KCNQ2 channels (as part of the M current) have been involved in neuronal excitability, resonance and behavior
Species:  Mouse
Tissue:  brain
References:  17
Phenotypes, Alleles and Disease Models Mouse data from MGI

Click here to show/hide data

Allele Composition & genetic background Accession Phenotype Id Phenotype Reference
Kcnq2tm1Dgen Kcnq2tm1Dgen/Kcnq2tm1Dgen
B6.129P2-Kcnq2/J
MGI:1309503  MP:0003412 abnormal afterhyperpolarization PMID: 19060215 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
MGI:1309503  MP:0004811 abnormal neuron physiology PMID: 18483067 
Kcnq2Nmf134 Kcnq2Nmf134/Kcnq2Nmf134
C57BL/6J
MGI:1309503  MP:0001650 abnormal seizure response to electrical stimulation
Kcnq2+|Kcnq2Nmf134 Kcnq2Nmf134/Kcnq2+
C57BL/6J
MGI:1309503  MP:0001650 abnormal seizure response to electrical stimulation
Kcnq2+|Kcnq2Nmf134 Kcnq2Nmf134/Kcnq2+
C57BL/6J-Kcnq2
MGI:1309503  MP:0001650 abnormal seizure response to electrical stimulation PMID: 16464983 
Kcnq2+|Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2+
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
MGI:1309503  MP:0001650 abnormal seizure response to electrical stimulation PMID: 18483067 
Kcnq2tm1Hsa Kcnq2tm1Hsa/Kcnq2tm1Hsa
involves: 129P2/OlaHsd * C57BL/6
MGI:1309503  MP:0001177 atelectasis PMID: 10854243 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
B6.129-Kcnq2
MGI:1309503  MP:0001265 decreased body size PMID: 18483067 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
FVB.129-Kcnq2
MGI:1309503  MP:0001265 decreased body size PMID: 18483067 
Kcnq2+|Kcnq2tm1Dgen Kcnq2tm1Dgen/Kcnq2+
B6.129P2-Kcnq2/J
MGI:1309503  MP:0009142 decreased prepulse inhibition PMID: 20592205 
Kcnq2+|Kcnq2tm1Hsa Kcnq2tm1Hsa/Kcnq2+
involves: 129P2/OlaHsd * C57BL/6
MGI:1309503  MP:0002906 increased susceptibility to pharmacologically induced seizures PMID: 10854243 
Kcnq2+|Kcnq2tm1Dgen Kcnq2tm1Dgen/Kcnq2+
involves: 129P2/OlaHsd * C57BL/6
MGI:1309503  MP:0002906 increased susceptibility to pharmacologically induced seizures
Kcnq2+|Kcnq2Nmf134 Kcnq2Nmf134/Kcnq2+
C57BL/6J-Kcnq2
MGI:1309503  MP:0002906 increased susceptibility to pharmacologically induced seizures PMID: 16464983 
Kcnq2tm1Hsa Kcnq2tm1Hsa/Kcnq2tm1Hsa
involves: 129P2/OlaHsd * C57BL/6
MGI:1309503  MP:0002058 neonatal lethality PMID: 10854243 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
B6.129-Kcnq2
MGI:1309503  MP:0002058 neonatal lethality PMID: 18483067 
Kcnq2tm1Dgen Kcnq2tm1Dgen/Kcnq2tm1Dgen
involves: 129P2/OlaHsd * C57BL/6
MGI:1309503  MP:0002082 postnatal lethality
Kcnq2+|Kcnq2Nmf134|Tg(Eno2-Scn2a1*)Q54Mm Kcnq2Nmf134/Kcnq2+,Tg(Eno2-Scn2a1*)Q54Mm/0
involves: C57BL/6J * SJL/J
MGI:1309503  MGI:3793790  MP:0002082 postnatal lethality PMID: 16464983 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
MGI:1309503  MP:0002082 postnatal lethality PMID: 18483067 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
FVB.129-Kcnq2
MGI:1309503  MP:0002082 postnatal lethality PMID: 18483067 
Kcnq2+|Kcnq2Nmf134|Tg(Eno2-Scn2a1*)Q54Mm Kcnq2Nmf134/Kcnq2+,Tg(Eno2-Scn2a1*)Q54Mm/0
involves: C57BL/6J * SJL/J
MGI:1309503  MGI:3793790  MP:0002083 premature death PMID: 16464983 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
B6.129-Kcnq2
MGI:1309503  MP:0002083 premature death PMID: 18483067 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
FVB.129-Kcnq2
MGI:1309503  MP:0002083 premature death PMID: 18483067 
Kcnq2+|Kcnq2Nmf134|Tg(Eno2-Scn2a1*)Q54Mm Kcnq2Nmf134/Kcnq2+,Tg(Eno2-Scn2a1*)Q54Mm/0
involves: C57BL/6J * SJL/J
MGI:1309503  MGI:3793790  MP:0002064 seizures PMID: 16464983 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
MGI:1309503  MP:0002064 seizures PMID: 18483067 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
FVB.129-Kcnq2
MGI:1309503  MP:0002064 seizures PMID: 18483067 
Kcnq2+|Kcnq2Nmf134|Tg(Eno2-Scn2a1*)Q54Mm Kcnq2Nmf134/Kcnq2+,Tg(Eno2-Scn2a1*)Q54Mm/0
involves: C57BL/6J * SJL/J
MGI:1309503  MGI:3793790  MP:0003997 tonic-clonic seizures PMID: 16464983 
Kcnq2tm1.1Naas Kcnq2tm1.1Naas/Kcnq2tm1.1Naas
B6.129-Kcnq2
MGI:1309503  MP:0003997 tonic-clonic seizures PMID: 18483067 
Clinically-Relevant Mutations and Pathophysiology
Disease:  myokymia with neonatal epilepsy
OMIM: 
Role: 
Comments: 
References:  7
Click column headers to sort
Type Species Molecular location Description Reference
Missense Human R207W ( in the S4 Voltage sensor segment) 7
Disease:  benign familial neonatal convulsions type 1 (BFNC1); also known as epilepsy, benign neonatal type 1 (EBN1)
OMIM: 
Orphanet: 
Role: 
Drugs: 
Side effects:  Retigabine: chills, pain, symptomatic hypotension, dizziness, nausea, myalgia, sweating, vomiting, asthenia and somnolence. Flupirtine: drowsiness, dizziness, dry mouth, pruritis and nausea
Therapeutic use:  Retigabine and flupirtine have shown antiepileptic activity in humans and in a broad range of seizure models in rodents. Retigabine and flupirtine may possess actions unrelated to KCNQ opening. It is unclear, therefore, if the efficacy of retigabine and flupirtine in animals models of epilepsy and pain and in human studies is entirely due to KCNQ activation.
Comments: 
References:  1,3-4,22
Click column headers to sort
Type Species Molecular location Description Reference
Deletion Human 3
Missense Human several 22
Disease:  Epileptic encephalopathy, early infantile, 7; EIEE7
OMIM: 
Orphanet: 
References: 
Mutations not determined
Disease:  Benign familial infantile seizures
Orphanet: 
References: 
Mutations not determined
Biologically Significant Variants
Isoform 2 is preferentially expressed in differentiated neurons
Protein accession:  O43526 
Amino acids:  872
Type:  Splice variant
Species:  Human
References:  23
soform 6 expression is prominent in fetal brain, undifferentiated neuroblastoma cells, and brain tumors
Protein accession:  O43526 
Amino acids:  13
Type:  Splice variant
Species:  Human
References:  23
General Comments
The M current is a slowly activating and deactivating potassium conductance that plays a critical role in determining the sub-threshold excitability of neurones as well as the responsiveness to synaptic inputs. The M current was first described in peripheral sympathetic neurones, and differential expression of this conductance produces subtypes of sympathetic neurones with distinct firing patterns. The M current is also expressed in many neurons in the central nervous system. The M-current is mediated by members of the Kv7 family, which form a heterotetrameric channel consisting of KCNQ3 subunits associated with either KCNQ2 or KCNQ5 subunits. Expression of KCNQ2, KCNQ3 and KCNQ5 proteins mostly overlaps with distribution of M current.

REFERENCES

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: Kv7.2. Last modified on 01/03/2012. Accessed on 24/05/2013. IUPHAR database (IUPHAR-DB), http://www.iuphar-db.org/DATABASE/ObjectDisplayForward?objectId=561.


Contact us | Print | Back to top | Help