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Protein Page:
Kv11.1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
Kv11.1 the ether-a-go-go related gene is a pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel is associated with cardiac arrhythmias and rhythmic excitability of the pituitary. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). 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 Kv11.2 and Kv11.3. Interacts with ALG10B. Heteromultimer with KCNE1 and KCNE2. Defects in Kv11.1 are the cause of long QT syndrome type 2 (LQT2), a heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress. Deafness is often associated with LQT2. Defects in Kv11.1 are the cause of short QT syndrome type 1 (SQT1), a heart disorder characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death. Four isoforms of the human protein are produced by alternative splicing. Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, multi-pass; Membrane protein, integral; Channel, potassium; Motility/polarity/chemotaxis
Cellular Component: voltage-gated potassium channel complex; cell surface; cytoplasm; plasma membrane; nuclear envelope
Molecular Function: identical protein binding; voltage-gated potassium channel activity; protein binding; protein homodimerization activity; ubiquitin protein ligase binding; delayed rectifier potassium channel activity; inward rectifier potassium channel activity; two-component sensor activity
Biological Process: synaptic transmission; regulation of membrane potential; two-component signal transduction system (phosphorelay); potassium ion homeostasis; regulation of the rate of heart contraction by hormone; cardiac muscle contraction
Reference #:  Q12809 (UniProtKB)
Alt. Names/Synonyms: Eag homolog; Eag-related protein 1; ERG; ERG-1; ERG1; Ether-a-go-go-related gene potassium channel 1; ether-a-go-go-related potassium channel protein; Ether-a-go-go-related protein 1; H-ERG; HERG; hERG-1; hERG1; KCNH2; Kv11.1; LQT2; Potassium voltage-gated channel subfamily H member 2; potassium voltage-gated channel, subfamily H (eag-related), member 2; SQT1; Voltage-gated potassium channel subunit Kv11.1; voltage-gated potassium channel, subfamily H, member 2
Gene Symbols: KCNH2
Molecular weight: 126,655 Da
Basal Isoelectric point: 8.2  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
Select Structure to View Below

Kv11.1

Protein Structure Not Found.


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Sites Implicated In
activity, induced: S283‑p, Y475‑p, Y611‑p, S890‑p, S1137‑p
activity, inhibited: S283‑p, S890‑p, T895‑p, S1137‑p
intracellular localization: S890‑p
molecular association, regulation: S283‑p, S1137‑p
protein stabilization: S890‑p

Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 3 S239-p RALVGPGsPPRSAPG
0 1 S243 GPGsPPRSAPGQLPs
0 1 S250-p SAPGQLPsPRAHSLN
0 1 S263 LNPDASGSSCSLART
0 1 S266 DASGSSCSLARTRSR
3 2 S283-p CASVRRAssADDIEA
0 3 S284-p ASVRRAssADDIEAM
0 5 S320-p SGLLNSTsDsDLVRY
0 1 S322-p LLNSTsDsDLVRYRT
0 2 S351 KGDPFLASPTSDREI
0 1 S354 PFLASPTSDREIIAP
0 1 - gap
0 1 - gap
0 1 T371 KERTHNVTEKVTQVL
0 1 K389 ADVLPEYKLQAPRIH
1 0 Y475-p LINFRTTyVNANEEV
1 1 Y611-p GPSIKDKyVTALYFT
0 4 S871-p DTNMIPGsPGSTELE
0 3 S874 MIPGsPGSTELEGGF
0 1 G879 PGSTELEGGFsRQRK
0 1 S882-p TELEGGFsRQRKRKL
4 3 S890-p RQRKRKLsFRRRtDK
4 1 T895-p KLsFRRRtDKDTEQP
1 3 K897 sFRRRtDKDTEQPGE
0 1 T899 RRRtDKDTEQPGEVS
0 3 Y1009-p GDSRGRQyQELPRCP
0 1 R1014 RQyQELPRCPAPTPS
0 2 S1029-p LLNIPLSsPGRRPRG
3 11 S1137-p EGPTRRLsLPGQLGA
  Kv11.1 iso2  
- gap
- gap
- under review  
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
S9-p AAPAGKAsRtGALRP
T11-p PAGKAsRtGALRPRA
S31-p RRAVRISsLVAQEVL
K49-ub ADVLPEYkLQAPRIH
Y135 LINFRTTYVNANEEV
Y271 GPSIKDKYVTALYFT
S531 DTNMIPGSPGSTELE
S534 MIPGSPGSTELEGGF
G539 PGSTELEGGFSRQRK
S542 TELEGGFSRQRKRKL
S550 RQRKRKLSFRRRTDK
T555 KLSFRRRTDKDTEQP
K557 SFRRRTDKDTEQPGE
T559 RRRTDKDTEQPGEVS
Y669 GDSRGRQYQELPRCP
R674 RQYQELPRCPAPTPS
S689 LLNIPLSSPGRRPRG
S797 EGPTRRLSLPGQLGA
  Kv11.1 iso5  
S239 RALVGPGSPPRSAPG
S243 GPGSPPRSAPGQLPS
S250 SAPGQLPSPRAHSLN
S263 LNPDASGSSCSLART
S266 DASGSSCSLARTRSR
S283 CASVRRASSADDIEA
S284 ASVRRASSADDIEAM
S320 SGLLNSTSDSDLVRY
S322 LLNSTSDSDLVRYRT
S351 KGDPFLASPTSDREI
S354 PFLASPTSDREIIAP
- gap
- gap
T371 KERTHNVTEKVTQVL
K389 ADVLPEYKLQAPRIH
Y475 LINFRTTYVNANEEV
Y611 GPSIKDKYVTALYFT
S871 DTNMIPGSPGSTELE
S874 MIPGSPGSTELEGGF
G879 PGSTELEGGFSRQRK
S882 TELEGGFSRQRKRKL
S890 RQRKRKLSFRRRtDt
T895-p KLSFRRRtDtDtEQP
T897-p SFRRRtDtDtEQPGE
T899-p RRRtDtDtEQPGEVS
Y1009 GDSRGRQYQELPRCP
R1014 RQYQELPRCPAPTPS
S1029 LLNIPLSSPGRRPRG
S1137 EGPTRRLSLPGQLGA
  mouse

 
S241-p LVGPGSAsPVAsIRG
S245-p GSAsPVAsIRGPHPS
S252 sIRGPHPSPRAQSLN
S265-p LNPDASGsSCsLART
S268-p DASGsSCsLARTRSR
S285-p CASVRRAssADDIEA
S286-p ASVRRAssADDIEAM
S322-p SGLLNSTsDSDLVRY
S324 LLNSTsDSDLVRYRT
S353-p KGDPFLAsPTsDREI
S356-p PFLAsPTsDREIIAP
- gap
- gap
T373 KERTHNVTEKVTQVL
K391 ADVLPEYKLQAPRIH
Y477 LINFRTTYVNANEEV
Y613 GPSIKDKYVTALYFT
S873-p DTNMIPGsPGsAELE
S876-p MIPGsPGsAELEsGF
S881-p PGsAELEsGFNRQRK
N884 AELEsGFNRQRKRKL
S892-p RQRKRKLsFRRRTDK
T897 KLsFRRRTDKDTEQP
K899 sFRRRTDKDTEQPGE
T901 RRRTDKDTEQPGEVS
Y1012 GDSRGRQYQELPrCP
R1017-m1 RQYQELPrCPAPAPS
S1032-p LLNIPLSsPGRRSRG
S1140-p DGPTRRLsLPGQLGA
  rat

 
S241 LVGPASASPVASIPG
S245 ASASPVASIPGPHPS
S252 SIPGPHPSPRAQSLN
S265 LNPDASGSSCSLART
S268 DASGSSCSLARTRSR
S285 CASVRRASSADDIEA
S286 ASVRRASSADDIEAM
S322 SGLLNSTSDSDLVRY
S324 LLNSTSDSDLVRYRT
S353 KGDPFLASPTSDREI
S356 PFLASPTSDREIIAP
- gap
- gap
T373 KERTHNVTEKVTQVL
K391 ADVLPEYKLQAPRIH
Y477 LINFRTTYVNANEEV
Y613-p GPSIKDKyVTALYFT
S873 DTNMIPGSPSSAELE
S876 MIPGSPSSAELESGF
S881 PSSAELESGFNRQRK
N884 AELESGFNRQRKRKL
S892 RQRKRKLSFRRRTDK
T897 KLSFRRRTDKDTEQP
K899 SFRRRTDKDTEQPGE
T901 RRRTDKDTEQPGEVS
Y1013 GDSRGRQYQELPRCP
R1018 RQYQELPRCPAPAPS
S1033 LLNIPLSSPGRRSRG
S1141 DGPTRRLSLPGQLGA
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