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Protein

Potassium voltage-gated channel subfamily H member 2

Gene

Kcnh2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionIon channel, Potassium channel, Voltage-gated channel
Biological processIon transport, Potassium transport, Transport
LigandPotassium

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Unplaced

Organism-specific databases

MGIiMGI:1341722 Kcnh2

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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

Keywords - Cellular componenti

Cell membrane, 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 residuei239PhosphoserineBy similarity1
Modified residuei241PhosphoserineCombined sources1
Modified residuei245PhosphoserineCombined sources1
Modified residuei285PhosphoserineCombined sources1
Modified residuei286PhosphoserineCombined sources1
Modified residuei322PhosphoserineCombined sources1
Modified residuei353PhosphoserineBy similarity1
Glycosylationi600N-linked (GlcNAc...) asparagineSequence analysis1
Modified residuei873PhosphoserineCombined sources1
Modified residuei876PhosphoserineCombined sources1
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.

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. Interacts with DNAJB12 and DNAJB14; chaperones DNAJB12 and DNAJB14 promote tetramerization (By similarity). Heteromultimer with KCNH6/ERG2 and KCNH7/ERG3 (By similarity). Interacts with ALG10B (By similarity). Heteromultimer with KCNE1 and KCNE2. Interacts with CANX. The core-glycosylated, but not the fully glycosylated form interacts with RNF207. Interacts with NDFIP1 and NDFIP2 (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

ComplexPortaliCPX-3197 Voltage-gated potassium channel complex variant 2
CPX-3198 Voltage-gated potassium channel complex variant 1
IntActiO35219, 31 interactors
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

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0498 Eukaryota
ENOG410XPSE LUCA
HOGENOMiHOG000230793
HOVERGENiHBG052232
InParanoidiO35219
KOiK04905
PhylomeDBiO35219

Family and domain databases

CDDicd00038 CAP_ED, 1 hit
cd00130 PAS, 1 hit
Gene3Di1.20.120.350, 1 hit
2.60.120.10, 1 hit
InterProiView protein in InterPro
IPR018490 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
IPR035965 PAS-like_dom_sf
IPR014710 RmlC-like_jellyroll
IPR027359 Volt_channel_dom_sf
PfamiView protein in Pfam
PF00027 cNMP_binding, 1 hit
PF00520 Ion_trans, 1 hit
PF13426 PAS_9, 1 hit
PRINTSiPR01463 EAGCHANLFMLY
PR01470 ERGCHANNEL
SMARTiView protein in SMART
SM00100 cNMP, 1 hit
SM00086 PAC, 1 hit
SUPFAMiSSF51206 SSF51206, 1 hit
SSF55785 SSF55785, 1 hit
TIGRFAMsiTIGR00229 sensory_box, 1 hit
PROSITEiView protein in PROSITE
PS50042 CNMP_BINDING_3, 1 hit
PS50113 PAC, 1 hit
PS50112 PAS, 1 hit

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.

This entry has 3 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q53Z09Q53Z09_MOUSE
Potassium channel erg1a
Kcnh2
1,162Annotation score:
A0A0R4J1K0A0A0R4J1K0_MOUSE
Potassium voltage-gated channel sub...
Kcnh2 mCG_16476
820Annotation score:

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

Similar proteinsi

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

ComplexPortaliCPX-3197 Voltage-gated potassium channel complex variant 2
CPX-3198 Voltage-gated potassium channel complex variant 1
IntActiO35219, 31 interactors
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

PROiPR:O35219
SOURCEiSearch...

Family and domain databases

CDDicd00038 CAP_ED, 1 hit
cd00130 PAS, 1 hit
Gene3Di1.20.120.350, 1 hit
2.60.120.10, 1 hit
InterProiView protein in InterPro
IPR018490 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
IPR035965 PAS-like_dom_sf
IPR014710 RmlC-like_jellyroll
IPR027359 Volt_channel_dom_sf
PfamiView protein in Pfam
PF00027 cNMP_binding, 1 hit
PF00520 Ion_trans, 1 hit
PF13426 PAS_9, 1 hit
PRINTSiPR01463 EAGCHANLFMLY
PR01470 ERGCHANNEL
SMARTiView protein in SMART
SM00100 cNMP, 1 hit
SM00086 PAC, 1 hit
SUPFAMiSSF51206 SSF51206, 1 hit
SSF55785 SSF55785, 1 hit
TIGRFAMsiTIGR00229 sensory_box, 1 hit
PROSITEiView protein in PROSITE
PS50042 CNMP_BINDING_3, 1 hit
PS50113 PAC, 1 hit
PS50112 PAS, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiKCNH2_MOUSE
AccessioniPrimary (citable) accession number: O35219
Secondary accession number(s): O35220, O35221, O35989
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 28, 2002
Last sequence update: November 28, 2002
Last modified: September 12, 2018
This is version 165 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
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