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Protein

Potassium voltage-gated channel subfamily E member 3

Gene

KCNE3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. Modulates the gating kinetics and enhances stability of the channel complex. Assembled with KCNB1 modulates the gating characteristics of the delayed rectifier voltage-dependent potassium channel KCNB1 (PubMed:12954870). Associated with KCNC4/Kv3.4 is proposed to form the subthreshold voltage-gated potassium channel in skeletal muscle and to establish the resting membrane potential (RMP) in muscle cells. Associated with KCNQ1/KCLQT1 may form the intestinal cAMP-stimulated potassium channel involved in chloride secretion that produces a current with nearly instantaneous activation with a linear current-voltage relationship.By similarity2 Publications

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Ion channel, Potassium channel, Voltage-gated channel

Keywords - Biological processi

Ion transport, Potassium transport, Transport

Keywords - Ligandi

Potassium

Enzyme and pathway databases

BioCyciZFISH:ENSG00000175538-MONOMER.
ReactomeiR-HSA-5576890. Phase 3 - rapid repolarisation.
R-HSA-5576893. Phase 2 - plateau phase.

Names & Taxonomyi

Protein namesi
Recommended name:
Potassium voltage-gated channel subfamily E member 3
Alternative name(s):
MinK-related peptide 2
Minimum potassium ion channel-related peptide 2
Potassium channel subunit beta MiRP2
Gene namesi
Name:KCNE3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

HGNCiHGNC:6243. KCNE3.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei58 – 78HelicalSequence analysisAdd BLAST21
Topological domaini79 – 103CytoplasmicSequence analysisAdd BLAST25

GO - Cellular componenti

  • cytoplasm Source: UniProtKB
  • dendrite Source: UniProtKB
  • membrane raft Source: UniProtKB
  • neuronal cell body membrane Source: UniProtKB
  • perikaryon Source: UniProtKB
  • vesicle Source: UniProtKB
  • voltage-gated potassium channel complex Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Cell projection, Cytoplasm, Membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi90D → N: Decreases current 4-fold in KCNH2/KCNE3 channel. 1 Publication1

Keywords - Diseasei

Brugada syndrome, Disease mutation

Organism-specific databases

DisGeNETi10008.
MalaCardsiKCNE3.
MIMi613119. phenotype.
OpenTargetsiENSG00000175538.
Orphaneti130. Brugada syndrome.
681. Hypokalemic periodic paralysis.
PharmGKBiPA393.

Polymorphism and mutation databases

BioMutaiKCNE3.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001442891 – 103Potassium voltage-gated channel subfamily E member 3Add BLAST103

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi5N-linked (GlcNAc...)Sequence analysis1
Glycosylationi22N-linked (GlcNAc...)Sequence analysis1
Glycosylationi41N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

PaxDbiQ9Y6H6.
PRIDEiQ9Y6H6.

PTM databases

iPTMnetiQ9Y6H6.
PhosphoSitePlusiQ9Y6H6.

Expressioni

Tissue specificityi

Expressed in hippocampal neurons (at protein level) (PubMed:12954870). Widely expressed with highest levels in kidney and moderate levels in small intestine.2 Publications

Gene expression databases

BgeeiENSG00000175538.
CleanExiHS_KCNE3.
ExpressionAtlasiQ9Y6H6. baseline and differential.
GenevisibleiQ9Y6H6. HS.

Organism-specific databases

HPAiHPA014849.

Interactioni

Subunit structurei

Interacts with KCNB1. Interacts with KCNC2 (By similarity). Associates with KCNC4/Kv3.4 (PubMed:11207363). Interacts with KCNQ1; produces a current with nearly instantaneous activation with a linear current-voltage relationship and alters membrane raft localization (By similarity) (PubMed:20533308).By similarity2 Publications

Protein-protein interaction databases

BioGridi115326. 10 interactors.
IntActiQ9Y6H6. 10 interactors.
MINTiMINT-8247512.
STRINGi9606.ENSP00000310557.

Structurei

Secondary structure

1103
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi8 – 29Combined sources22
Helixi55 – 80Combined sources26
Turni91 – 95Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2NDJNMR-A1-103[»]
ProteinModelPortaliQ9Y6H6.
SMRiQ9Y6H6.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the potassium channel KCNE family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiENOG410IZ1X. Eukaryota.
ENOG410XU3Y. LUCA.
GeneTreeiENSGT00390000008537.
HOGENOMiHOG000113209.
HOVERGENiHBG052228.
InParanoidiQ9Y6H6.
KOiK04897.
OMAiGPGPDNQ.
OrthoDBiEOG091G0Z41.
PhylomeDBiQ9Y6H6.
TreeFamiTF335981.

Family and domain databases

InterProiIPR000369. K_chnl_KCNE.
IPR005426. K_chnl_volt-dep_bsu_KCNE3.
[Graphical view]
PANTHERiPTHR15282:SF6. PTHR15282:SF6. 1 hit.
PfamiPF02060. ISK_Channel. 1 hit.
[Graphical view]
PRINTSiPR01606. KCNE3CHANNEL.
PR00168. KCNECHANNEL.

Sequencei

Sequence statusi: Complete.

Q9Y6H6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
METTNGTETW YESLHAVLKA LNATLHSNLL CRPGPGLGPD NQTEERRASL
60 70 80 90 100
PGRDDNSYMY ILFVMFLFAV TVGSLILGYT RSRKVDKRSD PYHVYIKNRV

SMI
Length:103
Mass (Da):11,710
Last modified:November 1, 1999 - v1
Checksum:i5235385E8D08BF10
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0586354T → A.1 PublicationCorresponds to variant rs200856070dbSNPEnsembl.1
Natural variantiVAR_05863639P → R.1 PublicationCorresponds to variant rs34604640dbSNPEnsembl.1
Natural variantiVAR_01506483R → H in some patients with periodic paralysis; unknown pathological significance; alters voltage dependence, lowers current and diminishes open probability in KCNC4/KCNE3 channel; lowers current in KCNQ1/KCNE3 channel. 2 PublicationsCorresponds to variant rs17215437dbSNPEnsembl.1
Natural variantiVAR_05863799R → H in BRGDA6; also in a patient suffering from drug-induced torsades de pointes. 2 PublicationsCorresponds to variant rs121908441dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF076531 mRNA. Translation: AAD28089.1.
AF302494 mRNA. Translation: AAG16255.1.
BC032235 mRNA. No translation available.
CCDSiCCDS8232.1.
RefSeqiNP_005463.1. NM_005472.4.
XP_016872536.1. XM_017017047.1.
XP_016872537.1. XM_017017048.1.
XP_016872538.1. XM_017017049.1.
XP_016872539.1. XM_017017050.1.
XP_016872540.1. XM_017017051.1.
XP_016872541.1. XM_017017052.1.
UniGeneiHs.523899.

Genome annotation databases

EnsembliENST00000310128; ENSP00000310557; ENSG00000175538.
ENST00000525550; ENSP00000433633; ENSG00000175538.
GeneIDi10008.
KEGGihsa:10008.
UCSCiuc001ovc.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF076531 mRNA. Translation: AAD28089.1.
AF302494 mRNA. Translation: AAG16255.1.
BC032235 mRNA. No translation available.
CCDSiCCDS8232.1.
RefSeqiNP_005463.1. NM_005472.4.
XP_016872536.1. XM_017017047.1.
XP_016872537.1. XM_017017048.1.
XP_016872538.1. XM_017017049.1.
XP_016872539.1. XM_017017050.1.
XP_016872540.1. XM_017017051.1.
XP_016872541.1. XM_017017052.1.
UniGeneiHs.523899.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2NDJNMR-A1-103[»]
ProteinModelPortaliQ9Y6H6.
SMRiQ9Y6H6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115326. 10 interactors.
IntActiQ9Y6H6. 10 interactors.
MINTiMINT-8247512.
STRINGi9606.ENSP00000310557.

PTM databases

iPTMnetiQ9Y6H6.
PhosphoSitePlusiQ9Y6H6.

Polymorphism and mutation databases

BioMutaiKCNE3.

Proteomic databases

PaxDbiQ9Y6H6.
PRIDEiQ9Y6H6.

Protocols and materials databases

DNASUi10008.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000310128; ENSP00000310557; ENSG00000175538.
ENST00000525550; ENSP00000433633; ENSG00000175538.
GeneIDi10008.
KEGGihsa:10008.
UCSCiuc001ovc.4. human.

Organism-specific databases

CTDi10008.
DisGeNETi10008.
GeneCardsiKCNE3.
GeneReviewsiKCNE3.
HGNCiHGNC:6243. KCNE3.
HPAiHPA014849.
MalaCardsiKCNE3.
MIMi604433. gene.
613119. phenotype.
neXtProtiNX_Q9Y6H6.
OpenTargetsiENSG00000175538.
Orphaneti130. Brugada syndrome.
681. Hypokalemic periodic paralysis.
PharmGKBiPA393.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IZ1X. Eukaryota.
ENOG410XU3Y. LUCA.
GeneTreeiENSGT00390000008537.
HOGENOMiHOG000113209.
HOVERGENiHBG052228.
InParanoidiQ9Y6H6.
KOiK04897.
OMAiGPGPDNQ.
OrthoDBiEOG091G0Z41.
PhylomeDBiQ9Y6H6.
TreeFamiTF335981.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000175538-MONOMER.
ReactomeiR-HSA-5576890. Phase 3 - rapid repolarisation.
R-HSA-5576893. Phase 2 - plateau phase.

Miscellaneous databases

ChiTaRSiKCNE3. human.
GeneWikiiKCNE3.
GenomeRNAii10008.
PROiQ9Y6H6.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000175538.
CleanExiHS_KCNE3.
ExpressionAtlasiQ9Y6H6. baseline and differential.
GenevisibleiQ9Y6H6. HS.

Family and domain databases

InterProiIPR000369. K_chnl_KCNE.
IPR005426. K_chnl_volt-dep_bsu_KCNE3.
[Graphical view]
PANTHERiPTHR15282:SF6. PTHR15282:SF6. 1 hit.
PfamiPF02060. ISK_Channel. 1 hit.
[Graphical view]
PRINTSiPR01606. KCNE3CHANNEL.
PR00168. KCNECHANNEL.
ProtoNetiSearch...

Entry informationi

Entry nameiKCNE3_HUMAN
AccessioniPrimary (citable) accession number: Q9Y6H6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: November 1, 1999
Last modified: November 30, 2016
This is version 137 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Caution

Variant His-83 has been associated with periodic paralysis (PubMed:11207363 and PubMed:12414843). The association could not be confirmed by further studies leading to the conclusion that His-83 does not play a causative role in the disease (PubMed:14504341 and PubMed:15037716).Curated

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.