KCNH2_HUMAN - dbPTM
KCNH2_HUMAN - PTM Information in dbPTM
Basic Information of Protein
UniProt ID KCNH2_HUMAN
UniProt AC Q12809
Protein Name Potassium voltage-gated channel subfamily H member 2
Gene Name KCNH2
Organism Homo sapiens (Human).
Sequence Length 1159
Subcellular Localization Cell membrane
Multi-pass membrane protein .
Protein Description 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). [PubMed: 18559421]
Protein Sequence MPVRRGHVAPQNTFLDTIIRKFEGQSRKFIIANARVENCAVIYCNDGFCELCGYSRAEVMQRPCTCDFLHGPRTQRRAAAQIAQALLGAEERKVEIAFYRKDGSCFLCLVDVVPVKNEDGAVIMFILNFEVVMEKDMVGSPAHDTNHRGPPTSWLAPGRAKTFRLKLPALLALTARESSVRSGGAGGAGAPGAVVVDVDLTPAAPSSESLALDEVTAMDNHVAGLGPAEERRALVGPGSPPRSAPGQLPSPRAHSLNPDASGSSCSLARTRSRESCASVRRASSADDIEAMRAGVLPPPPRHASTGAMHPLRSGLLNSTSDSDLVRYRTISKIPQITLNFVDLKGDPFLASPTSDREIIAPKIKERTHNVTEKVTQVLSLGADVLPEYKLQAPRIHRWTILHYSPFKAVWDWLILLLVIYTAVFTPYSAAFLLKETEEGPPATECGYACQPLAVVDLIVDIMFIVDILINFRTTYVNANEEVVSHPGRIAVHYFKGWFLIDMVAAIPFDLLIFGSGSEELIGLLKTARLLRLVRVARKLDRYSEYGAAVLFLLMCTFALIAHWLACIWYAIGNMEQPHMDSRIGWLHNLGDQIGKPYNSSGLGGPSIKDKYVTALYFTFSSLTSVGFGNVSPNTNSEKIFSICVMLIGSLMYASIFGNVSAIIQRLYSGTARYHTQMLRVREFIRFHQIPNPLRQRLEEYFQHAWSYTNGIDMNAVLKGFPECLQADICLHLNRSLLQHCKPFRGATKGCLRALAMKFKTTHAPPGDTLVHAGDLLTALYFISRGSIEILRGDVVVAILGKNDIFGEPLNLYARPGKSNGDVRALTYCDLHKIHRDDLLEVLDMYPEFSDHFWSSLEITFNLRDTNMIPGSPGSTELEGGFSRQRKRKLSFRRRTDKDTEQPGEVSALGPGRAGAGPSSRGRPGGPWGESPSSGPSSPESSEDEGPGRSSSPLRLVPFSSPRPPGEPPGGEPLMEDCEKSSDTCNPLSGAFSGVSNIFSFWGDSRGRQYQELPRCPAPTPSLLNIPLSSPGRRPRGDVESRLDALQRQLNRLETRLSADMATVLQLLQRQMTLVPPAYSAVTTPGPGPTSTSPLLPVSPLPTLTLDSLSQVSQFMACEELPPGAPELPQEGPTRRLSLPGQLGALTSQPLHRHGSDPGS
Overview of Protein Modification Sites with Functional and Structural Information
Experimental Post-Translational Modification Sites

* ASA = Accessible Surface Area

Locations Modification Substrate Peptides
&
Secondary Structure		 ASA (%) Reference Orthologous
Protein Cluster
2 (in isoform 7)Phosphorylation-39.3629116813
5 (in isoform 7)Phosphorylation-39.5429116813
9 (in isoform 2)Phosphorylation-8.35-
9 (in isoform 6)Phosphorylation-8.35-
11 (in isoform 2)Phosphorylation-52.43-
11 (in isoform 6)Phosphorylation-52.43-
30 (in isoform 2)Phosphorylation-3.0526434776
30 (in isoform 6)Phosphorylation-3.0526434776
31 (in isoform 2)Phosphorylation-2.4526434776
31 (in isoform 6)Phosphorylation-2.4526434776
39 (in isoform 2)Phosphorylation-1.7026434776
39 (in isoform 6)Phosphorylation-1.7026434776
49 (in isoform 2)Ubiquitination-4.83-
70UbiquitinationPCTCDFLHGPRTQRR
CCCCCCCCCHHHHHH		44.18-
166UbiquitinationRAKTFRLKLPALLAL
CCCCHHHHHHHHHHH		48.44-
179PhosphorylationALTARESSVRSGGAG
HHHHCHHHHHCCCCC		19.45-
239PhosphorylationRALVGPGSPPRSAPG
HHCCCCCCCCCCCCC		34.5629900121
243PhosphorylationGPGSPPRSAPGQLPS
CCCCCCCCCCCCCCC		44.5923312004
248UbiquitinationPRSAPGQLPSPRAHS
CCCCCCCCCCCCHHC		5.86-
250PhosphorylationSAPGQLPSPRAHSLN
CCCCCCCCCCHHCCC		35.2923403867
255PhosphorylationLPSPRAHSLNPDASG
CCCCCHHCCCCCCCC		28.9330177828
261PhosphorylationHSLNPDASGSSCSLA
HCCCCCCCCCCCHHH		46.7327732954
263PhosphorylationLNPDASGSSCSLART
CCCCCCCCCCHHHHH		26.1927732954
264PhosphorylationNPDASGSSCSLARTR
CCCCCCCCCHHHHHC		15.9627732954
266PhosphorylationDASGSSCSLARTRSR
CCCCCCCHHHHHCCH		27.3227732954
283PhosphorylationCASVRRASSADDIEA
HHHHHHCCCHHHHHH		25.0110837251
284PhosphorylationASVRRASSADDIEAM
HHHHHCCCHHHHHHH		34.4327732954
287 (in isoform 4)Ubiquitination-40.10-
293UbiquitinationDDIEAMRAGVLPPPP
HHHHHHHCCCCCCCC		10.20-
304PhosphorylationPPPPRHASTGAMHPL
CCCCCCCCCCCCCCC		22.6524117733
305PhosphorylationPPPRHASTGAMHPLR
CCCCCCCCCCCCCCC		29.7324117733
318PhosphorylationLRSGLLNSTSDSDLV
CCCCCCCCCCCHHHC		29.2927732954
319PhosphorylationRSGLLNSTSDSDLVR
CCCCCCCCCCHHHCH		35.1627732954
320PhosphorylationSGLLNSTSDSDLVRY
CCCCCCCCCHHHCHH		34.6327732954
322PhosphorylationLLNSTSDSDLVRYRT
CCCCCCCHHHCHHEE		32.3927732954
332 (in isoform 4)Ubiquitination-55.33-
351PhosphorylationKGDPFLASPTSDREI
CCCCCCCCCCCCCCC		30.7523186163
353PhosphorylationDPFLASPTSDREIIA
CCCCCCCCCCCCCHH		40.5427732954
354PhosphorylationPFLASPTSDREIIAP
CCCCCCCCCCCCHHH		37.7527732954
388PhosphorylationGADVLPEYKLQAPRI
CCCCCCHHHCCCCCE		18.3025884760
389UbiquitinationADVLPEYKLQAPRIH
CCCCCHHHCCCCCEE		32.25-
475PhosphorylationLINFRTTYVNANEEV
HHHCCCCCCCCCCHH		7.3518617000
598N-linked_GlycosylationDQIGKPYNSSGLGGP
HHCCCCCCCCCCCCC		37.9212063277
598N-linked_GlycosylationDQIGKPYNSSGLGGP
HHCCCCCCCCCCCCC		37.9212063277
611PhosphorylationGPSIKDKYVTALYFT
CCCHHHCCCCEEEEE		17.1118617000
623PhosphorylationYFTFSSLTSVGFGNV
EEEHHCCCCCCCCCC		24.2626074081
624PhosphorylationFTFSSLTSVGFGNVS
EEHHCCCCCCCCCCC		26.0726074081
645UbiquitinationKIFSICVMLIGSLMY
HHHHHHHHHHHHHHH		1.63-
661UbiquitinationSIFGNVSAIIQRLYS
HHHCCHHHHHHHHHC		9.91-
675PhosphorylationSGTARYHTQMLRVRE
CCCCHHHHHHHHHHH		13.5124719451
741UbiquitinationRSLLQHCKPFRGATK
HHHHHHCCCCCCCCH		45.10-
757UbiquitinationCLRALAMKFKTTHAP
HHHHHHHHHCCCCCC		37.79-
760PhosphorylationALAMKFKTTHAPPGD
HHHHHHCCCCCCCCC		27.2624719451
761PhosphorylationLAMKFKTTHAPPGDT
HHHHHCCCCCCCCCC		19.3424719451
768PhosphorylationTHAPPGDTLVHAGDL
CCCCCCCCEEEHHHH		36.2723898821
777PhosphorylationVHAGDLLTALYFISR
EEHHHHHHHHHHHHH		23.1623898821
780PhosphorylationGDLLTALYFISRGSI
HHHHHHHHHHHHCCE		9.0824719451
783PhosphorylationLTALYFISRGSIEIL
HHHHHHHHHCCEEEE		22.2323898821
801UbiquitinationVVVAILGKNDIFGEP
EEEEEECCCCCCCCC		48.26-
827PhosphorylationGDVRALTYCDLHKIH
CCCEEEEEEECCHHC		5.7025884760
840 (in isoform 4)Ubiquitination-48.62-
865PhosphorylationITFNLRDTNMIPGSP
EEEEECCCCCCCCCC		21.9126434776
871PhosphorylationDTNMIPGSPGSTELE
CCCCCCCCCCCCCCC		22.2727732954
874PhosphorylationMIPGSPGSTELEGGF
CCCCCCCCCCCCCCC		23.3027732954
875PhosphorylationIPGSPGSTELEGGFS
CCCCCCCCCCCCCCC		50.9727732954
882PhosphorylationTELEGGFSRQRKRKL
CCCCCCCCHHHHHHC		30.6727732954
890PhosphorylationRQRKRKLSFRRRTDK
HHHHHHCCCCCCCCC		21.5322653970
895PhosphorylationKLSFRRRTDKDTEQP
HCCCCCCCCCCCCCC		45.6610837251
959PhosphorylationPLRLVPFSSPRPPGE
CCEEEECCCCCCCCC		33.1027174698
960PhosphorylationLRLVPFSSPRPPGEP
CEEEECCCCCCCCCC		25.8827174698
1009PhosphorylationGDSRGRQYQELPRCP
CCCCCCCCCCCCCCC		11.55-
1014MethylationRQYQELPRCPAPTPS
CCCCCCCCCCCCCCH		52.23-
1019PhosphorylationLPRCPAPTPSLLNIP
CCCCCCCCCHHCCCC		27.8423312004
1021PhosphorylationRCPAPTPSLLNIPLS
CCCCCCCHHCCCCCC		48.2223312004
1028PhosphorylationSLLNIPLSSPGRRPR
HHCCCCCCCCCCCCC		29.1827732954
1029PhosphorylationLLNIPLSSPGRRPRG
HCCCCCCCCCCCCCC		38.7727732954
1040PhosphorylationRPRGDVESRLDALQR
CCCCCHHHHHHHHHH		37.6828188228
1137PhosphorylationEGPTRRLSLPGQLGA
CCCCCCCCCCCCHHH		30.6112431979
1146PhosphorylationPGQLGALTSQPLHRH
CCCHHHHCCCCCHHC		25.4523186163
1155PhosphorylationQPLHRHGSDPGS---
CCCHHCCCCCCC---		34.2125921289
Upstream regulatory proteins (kinases for phosphorylation sites, E3 ubiquitin ligases of ubiquitination sites, ...)
Modified Location Modified Residue Modification Type of Upstream Proteins Gene Name of Upstream Proteins UniProt AC of Upstream Proteins Sources
283SPhosphorylationKinasePRKACAP17612
GPS
283SPhosphorylationKinasePKA-FAMILY-GPS
283SPhosphorylationKinasePKA_GROUP-PhosphoELM
284SPhosphorylationKinasePRKD1Q15139
PSP
475YPhosphorylationKinaseSRC-FAMILY-GPS
611YPhosphorylationKinaseEGFRP00533
GPS
611YPhosphorylationKinaseSRC-FAMILY-GPS
890SPhosphorylationKinasePRKACAP17612
GPS
890SPhosphorylationKinasePRKCAP17252
GPS
895TPhosphorylationKinasePRKACAP17612
GPS
1137SPhosphorylationKinasePRKACAP17612
GPS
1137SPhosphorylationKinasePKA-FAMILY-GPS
1137SPhosphorylationKinasePKA_GROUP-PhosphoELM
-KUbiquitinationE3 ubiquitin ligaseSPOPO43791
PMID:26344095
-KUbiquitinationE3 ubiquitin ligaseNEDD4LQ96PU5
PMID:21463633
Functions of PTM Sites
Modified Location Modified Residue Modification Function Reference

Oops, there are no descriptions of PTM sites of KCNH2_HUMAN !!
Disease-associated PTM Sites based on SAP

* Distance = the distance between SAP position and PTM sites.

Modified Location Modification Variant Position
(Distance <= 10) 		Residue Change SAP Related Disease Reference

Oops, there are no SNP-PTM records of KCNH2_HUMAN !!
Protein-Protein Interaction
Interacting Protein Gene Name Interaction Type PPI Reference Domain-Domain Interactions
CDC73_HUMANCDC73physical
16169070
NDUS6_HUMANNDUFS6physical
16169070
1433E_HUMANYWHAEphysical
11953308
CAV3_HUMANCAV3physical
22879586
NED4L_HUMANNEDD4Lphysical
22879586
HSP7C_HUMANHSPA8physical
17569659
HS90A_HUMANHSP90AA1physical
17569659
DNJA1_HUMANDNAJA1physical
17569659
STIP1_HUMANSTIP1physical
17569659
BAG2_HUMANBAG2physical
17569659
CALX_HUMANCANXphysical
17569659
FKBP8_HUMANFKBP8physical
17569659
RB11A_HUMANRAB11Aphysical
23589291
HS90A_HUMANHSP90AA1physical
23963841
CHIP_HUMANSTUB1physical
23963841
NED4L_HUMANNEDD4Lphysical
24688054
CHIP_HUMANSTUB1physical
27998983
HSP7C_HUMANHSPA8physical
27998983
RN139_HUMANRNF139physical
27998983
Drug and Disease Associations
Kegg Disease
H00720 Long QT syndrome, including: Romano-Ward syndrome; Jervell and Lange-Nielsen syndrome (JLNS)
H00725 Short QT syndrome
OMIM Disease
613688Long QT syndrome 2 (LQT2)
609620Short QT syndrome 1 (SQT1)
Kegg Drug
D00110 Cocaine (USP); Cocaine (TN)
D00521 Terfenadine (JAN/USAN/INN); Seldane (TN)
D00631 Bepridil hydrochloride hydrate (JAN); Bepridil hydrochloride (USAN); Vascor (TN)
D00636 Amiodarone hydrochloride (JP16/USAN); Ancaron (TN); Cordarone (TN)
D00638 Flecainide acetate (JP16/USP); Tambocor (TN)
D00647 Dofetilide (JAN/USAN/INN); Tikosyn (TN)
D00648 Ibutilide fumarate (USAN); Corvert (TN)
D01026 Sotalol hydrochloride (JAN/USP); Betapace (TN); Sorine (TN)
D01326 Aprindine hydrochloride (JP16/USAN); Aspenon (TN)
D01856 Nifekalant hydrochloride (JAN); MS 551; Shinbit (TN)
D02182 Cocaine hydrochloride (JP16/USP); Cocaine hydrochloride (TN)
D02537 Dronedarone (INN)
D02910 Amiodarone (USAN/INN)
D02969 Aprindine (USAN/INN)
D03037 Azimilide dihydrochloride (USAN)
D03547 Clofilium phosphate (USAN/INN)
D03732 Dexsotalol hydrochloride (USAN)
D03914 Dronedarone hydrochloride (USAN); Multaq (TN)
D04955 Sematilide hydrochloride (USAN)
D06206 Trecetilide fumarate (USAN)
D06652 Tedisamil (USAN)
D06653 Tedisamil sesquifumarate (USAN)
D07520 Bepridil (INN); Bepadin (TN)
D07962 Flecainide (INN)
D08060 Ibutilide (INN)
D08525 Sotalol (INN); Darob mite (TN)
D09757 Terikalant (INN)
D09758 Ambasilide (INN)
D09759 Trecetilide (INN)
DrugBank
DB00346Alfuzosin
DB01118Amiodarone
DB00276Amsacrine
DB00637Astemizole
DB01136Carvedilol
DB00477Chlorpromazine
DB00537Ciprofloxacin
DB00604Cisapride
DB01211Clarithromycin
DB00204Dofetilide
DB00590Doxazosin
DB01142Doxepin
DB04855Dronedarone
DB00199Erythromycin
DB01218Halofantrine
DB00308Ibutilide
DB00458Imipramine
DB01110Miconazole
DB01100Pimozide
DB00457Prazosin
DB01182Propafenone
DB00908Quinidine
DB06144Sertindole
DB00489Sotalol
DB01162Terazosin
DB00679Thioridazine
DB00661Verapamil
Regulatory Network of KCNH2_HUMAN

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Related Literatures of Post-Translational Modification
N-linked Glycosylation
ReferencePubMed
"Role of glycosylation in cell surface expression and stability ofHERG potassium channels.";
Gong Q., Anderson C.L., January C.T., Zhou Z.;
Am. J. Physiol. 283:H77-H84(2002).
Cited for: MUTAGENESIS OF ASN-598; ASN-629 AND SER-631, AND GLYCOSYLATION ATASN-598.
Phosphorylation
ReferencePubMed
"Quantitative phosphoproteomic analysis of T cell receptor signalingreveals system-wide modulation of protein-protein interactions.";
Mayya V., Lundgren D.H., Hwang S.-I., Rezaul K., Wu L., Eng J.K.,Rodionov V., Han D.K.;
Sci. Signal. 2:RA46-RA46(2009).
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-320, AND MASSSPECTROMETRY.

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