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Protein

Potassium voltage-gated channel subfamily H member 2

Gene

Kcnh2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr) (By similarity).By similarity

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi744 – 861cNMPAdd BLAST118

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

Names & Taxonomyi

Protein namesi
Recommended name:
Potassium voltage-gated channel subfamily H member 2
Alternative name(s):
Ether-a-go-go-related gene potassium channel 1
Short name:
ERG-1
Short name:
Eag-related protein 1
Short name:
Ether-a-go-go-related protein 1
Short name:
MERG
Voltage-gated potassium channel subunit Kv11.1
Gene namesi
Name:Kcnh2
Synonyms:Erg, Merg1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Unplaced

Organism-specific databases

MGIiMGI:1341722. Kcnh2.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 405CytoplasmicSequence analysisAdd BLAST405
Transmembranei406 – 426Helical; Name=Segment S1Sequence analysisAdd BLAST21
Topological domaini427 – 452ExtracellularSequence analysisAdd BLAST26
Transmembranei453 – 473Helical; Name=Segment S2Sequence analysisAdd BLAST21
Topological domaini474 – 497CytoplasmicSequence analysisAdd BLAST24
Transmembranei498 – 518Helical; Name=Segment S3Sequence analysisAdd BLAST21
Topological domaini519 – 522ExtracellularSequence analysis4
Transmembranei523 – 543Helical; Voltage-sensor; Name=Segment S4Sequence analysisAdd BLAST21
Topological domaini544 – 549CytoplasmicSequence analysis6
Transmembranei550 – 570Helical; Name=Segment S5Sequence analysisAdd BLAST21
Topological domaini571 – 613ExtracellularSequence analysisAdd BLAST43
Intramembranei614 – 634Pore-forming; Name=Segment H5Sequence analysisAdd BLAST21
Topological domaini635 – 640ExtracellularSequence analysis6
Transmembranei641 – 661Helical; Name=Segment S6Sequence analysisAdd BLAST21
Topological domaini662 – 1162CytoplasmicSequence analysisAdd BLAST501

GO - Cellular componenti

  • cell surface Source: MGI
  • cytoplasm Source: MGI
  • integral component of plasma membrane Source: GO_Central
  • nuclear envelope Source: MGI
  • perinuclear region of cytoplasm Source: MGI
  • plasma membrane Source: MGI
  • voltage-gated potassium channel complex Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000540001 – 1162Potassium voltage-gated channel subfamily H member 2Add BLAST1162

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei179PhosphoserineBy similarity1
Modified residuei241PhosphoserineCombined sources1
Modified residuei245PhosphoserineCombined sources1
Modified residuei285PhosphoserineCombined sources1
Modified residuei286PhosphoserineCombined sources1
Modified residuei322PhosphoserineCombined sources1
Modified residuei353PhosphoserineBy similarity1
Glycosylationi600N-linked (GlcNAc...)Sequence analysis1
Modified residuei873PhosphoserineCombined sources1
Modified residuei876PhosphoserineCombined sources1
Modified residuei892PhosphoserineBy similarity1
Modified residuei1017Omega-N-methylarginineCombined sources1
Modified residuei1140PhosphoserineCombined sources1

Post-translational modificationi

Phosphorylated on serine and threonine residues.By similarity

Keywords - PTMi

Glycoprotein, Methylation, Phosphoprotein

Proteomic databases

MaxQBiO35219.
PaxDbiO35219.
PeptideAtlasiO35219.
PRIDEiO35219.

PTM databases

iPTMnetiO35219.
PhosphoSitePlusiO35219.

Expressioni

Tissue specificityi

Isoform 1 is expressed in heart, brain and testis and at low levels in lung. Isoform 3 is expressed predominantly in heart. The expression of isoform 2 is low in all tissues tested.

Gene expression databases

BgeeiENSMUSG00000038319.

Interactioni

Subunit structurei

The potassium channel is probably composed of a homo- or heterotetrameric complex of pore-forming alpha subunits that can associate with modulating beta subunits. Heteromultimer with KCNH6/ERG2, KCNH7/ERG3, KCNE1 and KCNE2 (By similarity). Interacts with ALG10B (By similarity). Interacts with CANX. The core-glycosylated, but not the fully glycosylated form interacts with RNF207 (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000047705.

Structurei

3D structure databases

ProteinModelPortaliO35219.
SMRiO35219.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini17 – 88PASPROSITE-ProRule annotationAdd BLAST72
Domaini92 – 144PACPROSITE-ProRule annotationAdd BLAST53

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi626 – 631Selectivity filterBy similarity6

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi299 – 302Poly-Pro4

Domaini

The segment S4 is probably the voltage-sensor and is characterized by a series of positively charged amino acids at every third position.

Sequence similaritiesi

Contains 1 cyclic nucleotide-binding domain.PROSITE-ProRule annotation
Contains 1 PAC (PAS-associated C-terminal) domain.PROSITE-ProRule annotation
Contains 1 PAS (PER-ARNT-SIM) domain.PROSITE-ProRule annotation

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0498. Eukaryota.
ENOG410XPSE. LUCA.
HOGENOMiHOG000230793.
HOVERGENiHBG052232.
InParanoidiO35219.
KOiK04905.
PhylomeDBiO35219.

Family and domain databases

Gene3Di2.60.120.10. 2 hits.
InterProiIPR018490. cNMP-bd-like.
IPR000595. cNMP-bd_dom.
IPR005821. Ion_trans_dom.
IPR003938. K_chnl_volt-dep_EAG/ELK/ERG.
IPR003967. K_chnl_volt-dep_ERG.
IPR001610. PAC.
IPR000014. PAS.
IPR000700. PAS-assoc_C.
IPR014710. RmlC-like_jellyroll.
[Graphical view]
PfamiPF00027. cNMP_binding. 1 hit.
PF00520. Ion_trans. 1 hit.
PF13426. PAS_9. 1 hit.
[Graphical view]
PRINTSiPR01463. EAGCHANLFMLY.
PR01470. ERGCHANNEL.
SMARTiSM00100. cNMP. 1 hit.
SM00086. PAC. 1 hit.
[Graphical view]
SUPFAMiSSF51206. SSF51206. 1 hit.
SSF55785. SSF55785. 1 hit.
TIGRFAMsiTIGR00229. sensory_box. 1 hit.
PROSITEiPS50042. CNMP_BINDING_3. 1 hit.
PS50113. PAC. 1 hit.
PS50112. PAS. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Note: Experimental confirmation may be lacking for some isoforms.
Isoform 1 (identifier: O35219-1) [UniParc]FASTAAdd to basket
Also known as: 1A, A

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MPVRRGHVAP QNTFLDTIIR KFEGQSRKFI IANARVENCA VIYCNDGFCE
60 70 80 90 100
LCGYSRAEVM QRPCTCDFLH GPRTQRRAAA QIAQALLGAE ERKVEIAFYR
110 120 130 140 150
KDGSCFLCLV DVVPVKNEDG AVIMFILNFE VVMEKDMVGS PAHDTNHRGP
160 170 180 190 200
STSWLASGRA KTFRLKLPAL LALTARESSV RTGSMHSAGA PGAVVVDVDL
210 220 230 240 250
TPAAPSSESL ALDEVSAMDN HVAGLGPAEE RRALVGPGSA SPVASIRGPH
260 270 280 290 300
PSPRAQSLNP DASGSSCSLA RTRSRESCAS VRRASSADDI EAMRAGALPP
310 320 330 340 350
PPRHASTGAM HPLRSGLLNS TSDSDLVRYR TISKIPQITL NFVDLKGDPF
360 370 380 390 400
LASPTSDREI IAPKIKERTH NVTEKVTQVL SLGADVLPEY KLQAPRIHRW
410 420 430 440 450
TILHYSPFKA VWDWLILLLV IYTAVFTPYS AAFLLKETED GSQAPDCGYA
460 470 480 490 500
CQPLAVVDLI VDIMFIVDIL INFRTTYVNA NEEVVSHPGR IAVHYFKGWF
510 520 530 540 550
LIDMVAAIPF DLLIFGSGSE ELIGLLKTAR LLRLVRVARK LDRYSEYGAA
560 570 580 590 600
VLFLLMCTFA LIAHWLACIW YAIGNMEQPH MDSHIGWLHN LGDQIGKPYN
610 620 630 640 650
SSGLGGPSIK DKYVTALYFT FSSLTSVGFG NVSPNTNSEK IFSICVMLIG
660 670 680 690 700
SLMYASIFGN VSAIIQRLYS GTARYHTQML RVREFIRFHQ IPNPLRQRLE
710 720 730 740 750
EYFQHAWSYT NGIDMNAVLK GFPECLQADI CLHLNRSLLQ HCKPFRGATK
760 770 780 790 800
GCLRALAMKF KTTHAPPGDT LVHAGDLLTA LYFISRGSIE ILRGDVVVAI
810 820 830 840 850
LGKNDIFGEP LNLYARPGKS NGDVRALTYC DLHKIHRDDL LEVLDMYPEF
860 870 880 890 900
SDHFWSSLEI TFNLRDTNMI PGSPGSAELE SGFNRQRKRK LSFRRRTDKD
910 920 930 940 950
TEQPGEVSAL GQGPARVGPG PSCRGQPGGP WGESPSSGPS SPESSEDEGP
960 970 980 990 1000
GRSSSPLRLV PFSSPRPPGD PPGGEPLTED GEKSDTCNPL SGAFSGVSNI
1010 1020 1030 1040 1050
FSFWGDSRGR QYQELPRCPA PAPSLLNIPL SSPGRRSRGD VESRLDALQR
1060 1070 1080 1090 1100
QLNRLETRLS ADMATVLQLL QRQMTLVPPA YSAVTTPGPG PTSASPLLPV
1110 1120 1130 1140 1150
GPVPTLTLDS LSQVSQFVAF EELPAGAPEL PQDGPTRRLS LPGQLGALTS
1160
QPLHRHGSDP GS
Length:1,162
Mass (Da):126,886
Last modified:November 28, 2002 - v2
Checksum:iA9455F7F10B61E46
GO
Isoform 2 (identifier: O35219-2) [UniParc]FASTAAdd to basket
Also known as: 1A'

The sequence of this isoform differs from the canonical sequence as follows:
     1-59: Missing.

Show »
Length:1,103
Mass (Da):120,195
Checksum:i28C9E5D432132689
GO
Isoform 3 (identifier: O35219-3) [UniParc]FASTAAdd to basket
Also known as: 1B, B

The sequence of this isoform differs from the canonical sequence as follows:
     1-378: MPVRRGHVAP...THNVTEKVTQ → MAIPTGKESR...VRISSLVAQE

Show »
Length:820
Mass (Da):90,129
Checksum:iD86EB7D0F3ADF7C4
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural varianti186H → R in strain: BALB/c. 1 Publication1
Natural varianti455A → T in strain: BALB/c. 1 Publication1
Natural varianti752C → Y in strain: BALB/c. 1 Publication1
Natural varianti1006D → N in strain: BALB/c. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0009701 – 378MPVRR…EKVTQ → MAIPTGKESRTGALQPRAQK GRVRRAVRISSLVAQE in isoform 3. 2 PublicationsAdd BLAST378
Alternative sequenceiVSP_0009691 – 59Missing in isoform 2. 1 PublicationAdd BLAST59

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF012868 mRNA. Translation: AAC53418.1.
AF012869 mRNA. Translation: AAC53419.1.
AF012871, AF012870 Genomic DNA. Translation: AAC53420.1.
AF012871 Genomic DNA. Translation: AAC53421.1.
AF012871, AF012870 Genomic DNA. Translation: AAC53422.1.
AF034762 mRNA. Translation: AAB87571.1.
CCDSiCCDS19116.1. [O35219-1]
CCDS80224.1. [O35219-3]
RefSeqiNP_001281091.1. NM_001294162.1.
UniGeneiMm.6539.

Genome annotation databases

GeneIDi16511.
KEGGimmu:16511.
UCSCiuc008wrb.1. mouse. [O35219-3]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF012868 mRNA. Translation: AAC53418.1.
AF012869 mRNA. Translation: AAC53419.1.
AF012871, AF012870 Genomic DNA. Translation: AAC53420.1.
AF012871 Genomic DNA. Translation: AAC53421.1.
AF012871, AF012870 Genomic DNA. Translation: AAC53422.1.
AF034762 mRNA. Translation: AAB87571.1.
CCDSiCCDS19116.1. [O35219-1]
CCDS80224.1. [O35219-3]
RefSeqiNP_001281091.1. NM_001294162.1.
UniGeneiMm.6539.

3D structure databases

ProteinModelPortaliO35219.
SMRiO35219.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000047705.

PTM databases

iPTMnetiO35219.
PhosphoSitePlusiO35219.

Proteomic databases

MaxQBiO35219.
PaxDbiO35219.
PeptideAtlasiO35219.
PRIDEiO35219.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi16511.
KEGGimmu:16511.
UCSCiuc008wrb.1. mouse. [O35219-3]

Organism-specific databases

CTDi3757.
MGIiMGI:1341722. Kcnh2.

Phylogenomic databases

eggNOGiKOG0498. Eukaryota.
ENOG410XPSE. LUCA.
HOGENOMiHOG000230793.
HOVERGENiHBG052232.
InParanoidiO35219.
KOiK04905.
PhylomeDBiO35219.

Miscellaneous databases

PROiO35219.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000038319.

Family and domain databases

Gene3Di2.60.120.10. 2 hits.
InterProiIPR018490. cNMP-bd-like.
IPR000595. cNMP-bd_dom.
IPR005821. Ion_trans_dom.
IPR003938. K_chnl_volt-dep_EAG/ELK/ERG.
IPR003967. K_chnl_volt-dep_ERG.
IPR001610. PAC.
IPR000014. PAS.
IPR000700. PAS-assoc_C.
IPR014710. RmlC-like_jellyroll.
[Graphical view]
PfamiPF00027. cNMP_binding. 1 hit.
PF00520. Ion_trans. 1 hit.
PF13426. PAS_9. 1 hit.
[Graphical view]
PRINTSiPR01463. EAGCHANLFMLY.
PR01470. ERGCHANNEL.
SMARTiSM00100. cNMP. 1 hit.
SM00086. PAC. 1 hit.
[Graphical view]
SUPFAMiSSF51206. SSF51206. 1 hit.
SSF55785. SSF55785. 1 hit.
TIGRFAMsiTIGR00229. sensory_box. 1 hit.
PROSITEiPS50042. CNMP_BINDING_3. 1 hit.
PS50113. PAC. 1 hit.
PS50112. PAS. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiKCNH2_MOUSE
AccessioniPrimary (citable) accession number: O35219
Secondary accession number(s): O35220, O35221, O35989
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 28, 2002
Last sequence update: November 28, 2002
Last modified: November 2, 2016
This is version 155 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. 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.