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Protein

Potassium voltage-gated channel subfamily H member 6

Gene

KCNH6

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

Functioni

Pore-forming (alpha) subunit of voltage-gated potassium channel. Elicits a slowly activating, rectifying current (By similarity). Channel properties may be modulated by cAMP and subunit assembly.By similarity

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi594 – 711cNMPAdd BLAST118

GO - Molecular functioni

GO - Biological processi

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-HSA-1296072 Voltage gated Potassium channels

Names & Taxonomyi

Protein namesi
Recommended name:
Potassium voltage-gated channel subfamily H member 6
Alternative name(s):
Ether-a-go-go-related gene potassium channel 2
Short name:
ERG-2
Short name:
Eag-related protein 2
Short name:
Ether-a-go-go-related protein 2
Short name:
hERG-2
Short name:
hERG2
Voltage-gated potassium channel subunit Kv11.2
Gene namesi
Name:KCNH6
Synonyms:ERG2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

EuPathDBiHostDB:ENSG00000173826.14
HGNCiHGNC:18862 KCNH6
MIMi608168 gene
neXtProtiNX_Q9H252

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 – 261CytoplasmicSequence analysisAdd BLAST261
Transmembranei262 – 282Helical; Name=Segment S1Sequence analysisAdd BLAST21
Topological domaini283 – 298ExtracellularSequence analysisAdd BLAST16
Transmembranei299 – 319Helical; Name=Segment S2Sequence analysisAdd BLAST21
Topological domaini320 – 340CytoplasmicSequence analysisAdd BLAST21
Transmembranei341 – 361Helical; Name=Segment S3Sequence analysisAdd BLAST21
Topological domaini362 – 370ExtracellularSequence analysis9
Transmembranei371 – 391Helical; Voltage-sensor; Name=Segment S4Sequence analysisAdd BLAST21
Topological domaini392 – 398CytoplasmicSequence analysis7
Transmembranei399 – 419Helical; Name=Segment S5Sequence analysisAdd BLAST21
Topological domaini420 – 463ExtracellularSequence analysisAdd BLAST44
Intramembranei464 – 484Pore-forming; Name=Segment H5Sequence analysisAdd BLAST21
Topological domaini485 – 490ExtracellularSequence analysis6
Transmembranei491 – 511Helical; Name=Segment S6Sequence analysisAdd BLAST21
Topological domaini512 – 994CytoplasmicSequence analysisAdd BLAST483

Keywords - Cellular componenti

Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi81033
OpenTargetsiENSG00000173826
PharmGKBiPA38722

Chemistry databases

ChEMBLiCHEMBL2362996
DrugBankiDB00590 Doxazosin
DB00308 Ibutilide
DB01110 Miconazole
DB00457 Prazosin
DB01162 Terazosin
GuidetoPHARMACOLOGYi573

Polymorphism and mutation databases

BioMutaiKCNH6
DMDMi26006810

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000540131 – 994Potassium voltage-gated channel subfamily H member 6Add BLAST994

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi449N-linked (GlcNAc...) (complex) asparagine1 Publication1

Keywords - PTMi

Glycoprotein

Proteomic databases

PaxDbiQ9H252
PeptideAtlasiQ9H252
PRIDEiQ9H252
ProteomicsDBi80495
80496 [Q9H252-2]
80497 [Q9H252-3]

PTM databases

iPTMnetiQ9H252
PhosphoSitePlusiQ9H252

Expressioni

Tissue specificityi

Expressed in prolactin-secreting adenomas.1 Publication

Gene expression databases

BgeeiENSG00000173826
CleanExiHS_KCNH6
ExpressionAtlasiQ9H252 baseline and differential
GenevisibleiQ9H252 HS

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. Heteromultimers with KCNH2/ERG1 and KCNH7/ERG3 (By similarity).By similarity

Protein-protein interaction databases

STRINGi9606.ENSP00000463533

Structurei

3D structure databases

ProteinModelPortaliQ9H252
SMRiQ9H252
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini41 – 70PASAdd BLAST30
Domaini92 – 144PACAdd BLAST53

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi476 – 481Selectivity filterBy similarity6

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
GeneTreeiENSGT00900000140833
HOGENOMiHOG000230793
HOVERGENiHBG052232
InParanoidiQ9H252
KOiK04909
OMAiADSFWSK
OrthoDBiEOG091G024C
PhylomeDBiQ9H252
TreeFamiTF313130

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
IPR030172 KCNH6
IPR000014 PAS
IPR035965 PAS-like_dom_sf
IPR014710 RmlC-like_jellyroll
IPR027359 Volt_channel_dom_sf
PANTHERiPTHR10217:SF468 PTHR10217:SF468, 1 hit
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
SUPFAMiSSF51206 SSF51206, 1 hit
SSF55785 SSF55785, 1 hit
PROSITEiView protein in PROSITE
PS50042 CNMP_BINDING_3, 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.
Isoform 1 (identifier: Q9H252-1) [UniParc]FASTAAdd to basket

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 QNTYLDTIIR KFEGQSRKFL IANAQMENCA IIYCNDGFCE
60 70 80 90 100
LFGYSRVEVM QQPCTCDFLT GPNTPSSAVS RLAQALLGAE ECKVDILYYR
110 120 130 140 150
KDASSFRCLV DVVPVKNEDG AVIMFILNFE DLAQLLAKCS SRSLSQRLLS
160 170 180 190 200
QSFLGSEGSH GRPGGPGPGT GRGKYRTISQ IPQFTLNFVE FNLEKHRSSS
210 220 230 240 250
TTEIEIIAPH KVVERTQNVT EKVTQVLSLG ADVLPEYKLQ APRIHRWTIL
260 270 280 290 300
HYSPFKAVWD WLILLLVIYT AVFTPYSAAF LLSDQDESRR GACSYTCSPL
310 320 330 340 350
TVVDLIVDIM FVVDIVINFR TTYVNTNDEV VSHPRRIAVH YFKGWFLIDM
360 370 380 390 400
VAAIPFDLLI FRTGSDETTT LIGLLKTARL LRLVRVARKL DRYSEYGAAV
410 420 430 440 450
LFLLMCTFAL IAHWLACIWY AIGNVERPYL EHKIGWLDSL GVQLGKRYNG
460 470 480 490 500
SDPASGPSVQ DKYVTALYFT FSSLTSVGFG NVSPNTNSEK VFSICVMLIG
510 520 530 540 550
SLMYASIFGN VSAIIQRLYS GTARYHTQML RVKEFIRFHQ IPNPLRQRLE
560 570 580 590 600
EYFQHAWSYT NGIDMNAVLK GFPECLQADI CLHLHRALLQ HCPAFSGAGK
610 620 630 640 650
GCLRALAVKF KTTHAPPGDT LVHLGDVLST LYFISRGSIE ILRDDVVVAI
660 670 680 690 700
LGKNDIFGEP VSLHAQPGKS SADVRALTYC DLHKIQRADL LEVLDMYPAF
710 720 730 740 750
AESFWSKLEV TFNLRDAAGG LHSSPRQAPG SQDHQGFFLS DNQSGSPHEL
760 770 780 790 800
GPQFPSKGYS LLGPGSQNSM GAGPCAPGHP DAAPPLSISD ASGLWPELLQ
810 820 830 840 850
EMPPRHSPQS PQEDPDCWPL KLGSRLEQLQ AQMNRLESRV SSDLSRILQL
860 870 880 890 900
LQKPMPQGHA SYILEAPASN DLALVPIASE TTSPGPRLPQ GFLPPAQTPS
910 920 930 940 950
YGDLDDCSPK HRNSSPRMPH LAVATDKTLA PSSEQEQPEG LWPPLASPLH
960 970 980 990
PLEVQGLICG PCFSSLPEHL GSVPKQLDFQ RHGSDPGFAG SWGH
Length:994
Mass (Da):109,925
Last modified:March 1, 2001 - v1
Checksum:iBE9ECB349A798576
GO
Isoform 2 (identifier: Q9H252-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     419-472: WYAIGNVERPYLEHKIGWLDSLGVQLGKRYNGSDPASGPSVQDKYVTALYFTFS → C
     745-780: Missing.

Show »
Length:905
Mass (Da):100,450
Checksum:i0556E274946D2B7E
GO
Isoform 3 (identifier: Q9H252-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     501-502: SL → CE
     503-994: Missing.

Show »
Length:502
Mass (Da):56,298
Checksum:i6CBD9F336841809B
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti963F → L in BAC03764 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_053857165G → R. Corresponds to variant dbSNP:rs35399062Ensembl.1
Natural variantiVAR_053858925T → M1 PublicationCorresponds to variant dbSNP:rs35819807Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_000977419 – 472WYAIG…YFTFS → C in isoform 2. 1 PublicationAdd BLAST54
Alternative sequenceiVSP_000979501 – 502SL → CE in isoform 3. 1 Publication2
Alternative sequenceiVSP_000980503 – 994Missing in isoform 3. 1 PublicationAdd BLAST492
Alternative sequenceiVSP_000978745 – 780Missing in isoform 2. 1 PublicationAdd BLAST36

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF311913 mRNA Translation: AAG40871.1
AK090969 mRNA Translation: BAC03559.1
AK091877 mRNA Translation: BAC03764.1
BC006334 mRNA Translation: AAH06334.1
CCDSiCCDS11638.1 [Q9H252-1]
CCDS11639.1 [Q9H252-2]
RefSeqiNP_001265848.1, NM_001278919.1
NP_001265849.1, NM_001278920.1
NP_110406.1, NM_030779.3 [Q9H252-1]
NP_775115.1, NM_173092.2 [Q9H252-2]
XP_011523612.1, XM_011525310.2 [Q9H252-2]
XP_016880664.1, XM_017025175.1 [Q9H252-1]
UniGeneiHs.591177

Genome annotation databases

EnsembliENST00000456941; ENSP00000396900; ENSG00000173826 [Q9H252-2]
ENST00000580652; ENSP00000464672; ENSG00000173826 [Q9H252-3]
ENST00000581784; ENSP00000463830; ENSG00000173826 [Q9H252-2]
ENST00000583023; ENSP00000463533; ENSG00000173826 [Q9H252-1]
GeneIDi81033
KEGGihsa:81033
UCSCiuc002jax.2 human [Q9H252-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiKCNH6_HUMAN
AccessioniPrimary (citable) accession number: Q9H252
Secondary accession number(s): Q9BRD7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 28, 2002
Last sequence update: March 1, 2001
Last modified: June 20, 2018
This is version 163 of the entry and version 1 of the sequence. See 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

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 17
    Human chromosome 17: 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. SIMILARITY comments
    Index of protein domains and families

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