KCNQ1_HUMAN - dbPTM
KCNQ1_HUMAN - PTM Information in dbPTM
Basic Information of Protein
UniProt ID KCNQ1_HUMAN
UniProt AC P51787
Protein Name Potassium voltage-gated channel subfamily KQT member 1 {ECO:0000305}
Gene Name KCNQ1 {ECO:0000312|HGNC:HGNC:6294}
Organism Homo sapiens (Human).
Sequence Length 676
Subcellular Localization Cell membrane
Multi-pass membrane protein . Cytoplasmic vesicle membrane . Early endosome . Membrane raft . Endoplasmic reticulum . Basolateral cell membrane . Colocalized with KCNE3 at the plasma membrane (PubMed:10646604). Upon 17beta-oestradiol
Protein Description Potassium channel that plays an important role in a number of tissues, including heart, inner ear, stomach and colon (By similarity). [PubMed: 10646604 Associates with KCNE beta subunits that modulates current kinetics (By similarity)]
Protein Sequence MAAASSPPRAERKRWGWGRLPGARRGSAGLAKKCPFSLELAEGGPAGGALYAPIAPGAPGPAPPASPAAPAAPPVASDLGPRPPVSLDPRVSIYSTRRPVLARTHVQGRVYNFLERPTGWKCFVYHFAVFLIVLVCLIFSVLSTIEQYAALATGTLFWMEIVLVVFFGTEYVVRLWSAGCRSKYVGLWGRLRFARKPISIIDLIVVVASMVVLCVGSKGQVFATSAIRGIRFLQILRMLHVDRQGGTWRLLGSVVFIHRQELITTLYIGFLGLIFSSYFVYLAEKDAVNESGRVEFGSYADALWWGVVTVTTIGYGDKVPQTWVGKTIASCFSVFAISFFALPAGILGSGFALKVQQKQRQKHFNRQIPAAASLIQTAWRCYAAENPDSSTWKIYIRKAPRSHTLLSPSPKPKKSVVVKKKKFKLDKDNGVTPGEKMLTVPHITCDPPEERRLDHFSVDGYDSSVRKSPTLLEVSMPHFMRTNSFAEDLDLEGETLLTPITHISQLREHHRATIKVIRRMQYFVAKKKFQQARKPYDVRDVIEQYSQGHLNLMVRIKELQRRLDQSIGKPSLFISVSEKSKDRGSNTIGARLNRVEDKVTQLDQRLALITDMLHQLLSLHGGSTPGSGGPPREGGAHITQPCGSGGSVDPELFLPSNTLPTYEQLTVPRRGPDEGS
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
5Phosphorylation---MAAASSPPRAER
---CCCCCCCCHHHH
38.3523663014
6Phosphorylation--MAAASSPPRAERK
--CCCCCCCCHHHHH
33.9123663014
27PhosphorylationLPGARRGSAGLAKKC
CCCCCCCCHHHHHHC
19.8328857561
92PhosphorylationVSLDPRVSIYSTRRP
CCCCCCCEEEECCCC
19.6526657352
94PhosphorylationLDPRVSIYSTRRPVL
CCCCCEEEECCCCEE
9.1123312004
95PhosphorylationDPRVSIYSTRRPVLA
CCCCEEEECCCCEEE
17.48-
96PhosphorylationPRVSIYSTRRPVLAR
CCCEEEECCCCEEEE
17.46-
184PhosphorylationSAGCRSKYVGLWGRL
HCCCCCCCCCHHHHH
10.8622817900
199PhosphorylationRFARKPISIIDLIVV
HCCCCCCCHHHHHHH
23.24-
289N-linked_GlycosylationLAEKDAVNESGRVEF
HHHHHCCCCCCCCCC
39.16UniProtKB CARBOHYD
402PhosphorylationYIRKAPRSHTLLSPS
EEEECCCCCCCCCCC
21.5522210691
404PhosphorylationRKAPRSHTLLSPSPK
EECCCCCCCCCCCCC
30.5529978859
407PhosphorylationPRSHTLLSPSPKPKK
CCCCCCCCCCCCCCC
26.8328450419
409PhosphorylationSHTLLSPSPKPKKSV
CCCCCCCCCCCCCEE
42.0328450419
424UbiquitinationVVKKKKFKLDKDNGV
EEEECEEECCCCCCC
65.73-
439PhosphorylationTPGEKMLTVPHITCD
CCCCCEEECCEEECC
28.6124043423
444PhosphorylationMLTVPHITCDPPEER
EEECCEEECCCCHHH
14.0724043423
445S-nitrosylationLTVPHITCDPPEERR
EECCEEECCCCHHHC
7.9622178444
445S-nitrosocysteineLTVPHITCDPPEERR
EECCEEECCCCHHHC
7.96-
457PhosphorylationERRLDHFSVDGYDSS
HHCCCCCCCCCCCCC
18.8428348404
461PhosphorylationDHFSVDGYDSSVRKS
CCCCCCCCCCCCCCC
14.2724043423
463PhosphorylationFSVDGYDSSVRKSPT
CCCCCCCCCCCCCCC
23.4224043423
464PhosphorylationSVDGYDSSVRKSPTL
CCCCCCCCCCCCCCE
24.0422817900
468PhosphorylationYDSSVRKSPTLLEVS
CCCCCCCCCCEEEEE
16.7428857561
470PhosphorylationSSVRKSPTLLEVSMP
CCCCCCCCEEEEECC
51.5822817900
475PhosphorylationSPTLLEVSMPHFMRT
CCCEEEEECCCCCCC
19.7423312004
482PhosphorylationSMPHFMRTNSFAEDL
ECCCCCCCCCCCHHC
24.3528450419
484PhosphorylationPHFMRTNSFAEDLDL
CCCCCCCCCCHHCCC
25.8126657352
513PhosphorylationLREHHRATIKVIRRM
HHHHHHHHHHHHHHH
22.6822817900
577PhosphorylationPSLFISVSEKSKDRG
CEEEEEECHHHCCCC
32.3022817900
666PhosphorylationLPTYEQLTVPRRGPD
CCCCCCCCCCCCCCC
28.3224719451

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
27SPhosphorylationKinasePRKACAP17612
GPS
27SPhosphorylationKinasePKA-Uniprot
482TPhosphorylationKinaseCAMK2DQ13557
PSP
484SPhosphorylationKinaseCAMK2DQ13557
PSP

Functions of PTM Sites
Modified Location Modified Residue Modification Function Reference
27SPhosphorylation

11799244

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 KCNQ1_HUMAN !!

Protein-Protein Interaction
Interacting Protein Gene Name Interaction Type PPI Reference Domain-Domain Interactions
AKAP9_HUMANAKAP9physical
11799244
KAP2_HUMANPRKAR2Aphysical
11799244
PP1A_HUMANPPP1CAphysical
11799244
KAPCA_HUMANPRKACAphysical
11799244
KCNE1_HUMANKCNE1physical
11101505
KCNE2_HUMANKCNE2physical
11101505
KCNQ2_HUMANKCNQ2physical
12524525
HSP74_HUMANHSPA4physical
19114714
KCNE1_HUMANKCNE1physical
19114714
NEDD4_HUMANNEDD4physical
22024150
UBP2_HUMANUSP2physical
22024150
AP2M1_HUMANAP2M1physical
23529131
NED4L_HUMANNEDD4Lphysical
23529131
NED4L_HUMANNEDD4Lphysical
26405101
BACE1_HUMANBACE1physical
26454161
AKAP9_HUMANAKAP9physical
18093912
KCNE1_HUMANKCNE1physical
24713462
AKAP9_HUMANAKAP9physical
24713462

Drug and Disease Associations
Kegg Disease
H00409 Type II diabetes mellitus
H00720 Long QT syndrome, including: Romano-Ward syndrome; Jervell and Lange-Nielsen syndrome (JLNS)
H00725 Short QT syndrome
H00731 Atrial fibrillation
OMIM Disease
192500Long QT syndrome 1 (LQT1)
220400Jervell and Lange-Nielsen syndrome 1 (JLNS1)
607554Atrial fibrillation, familial, 3 (ATFB3)
609621Short QT syndrome 2 (SQT2)
125853Diabetes mellitus, non-insulin-dependent (NIDDM)
Kegg Drug
D00631 Bepridil hydrochloride hydrate (JAN); Bepridil hydrochloride (USAN); Vascor (TN)
D00636 Amiodarone hydrochloride (JP16/USAN); Ancaron (TN); Cordarone (TN)
D02537 Dronedarone (INN)
D02910 Amiodarone (USAN/INN)
D03037 Azimilide dihydrochloride (USAN)
D03914 Dronedarone hydrochloride (USAN); Multaq (TN)
D04741 Linopirdine (USAN/INN)
D06652 Tedisamil (USAN)
D06653 Tedisamil sesquifumarate (USAN)
D07520 Bepridil (INN); Bepadin (TN)
D09757 Terikalant (INN)
D09758 Ambasilide (INN)
DrugBank
DB01244Bepridil
DB00808Indapamide
Regulatory Network of KCNQ1_HUMAN

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Related Literatures of Post-Translational Modification

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