| 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 | ||
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* ASA = Accessible Surface Area
| Locations | Modification | Substrate Peptides & Secondary Structure |
ASA (%) | Reference | Orthologous Protein Cluster |
|---|---|---|---|---|---|
| 5 | Phosphorylation | ---MAAASSPPRAER ---CCCCCCCCHHHH | 38.35 | 23663014 | |
| 6 | Phosphorylation | --MAAASSPPRAERK --CCCCCCCCHHHHH | 33.91 | 23663014 | |
| 27 | Phosphorylation | LPGARRGSAGLAKKC CCCCCCCCHHHHHHC | 19.83 | 28857561 | |
| 92 | Phosphorylation | VSLDPRVSIYSTRRP CCCCCCCEEEECCCC | 19.65 | 26657352 | |
| 94 | Phosphorylation | LDPRVSIYSTRRPVL CCCCCEEEECCCCEE | 9.11 | 23312004 | |
| 95 | Phosphorylation | DPRVSIYSTRRPVLA CCCCEEEECCCCEEE | 17.48 | - | |
| 96 | Phosphorylation | PRVSIYSTRRPVLAR CCCEEEECCCCEEEE | 17.46 | - | |
| 184 | Phosphorylation | SAGCRSKYVGLWGRL HCCCCCCCCCHHHHH | 10.86 | 22817900 | |
| 199 | Phosphorylation | RFARKPISIIDLIVV HCCCCCCCHHHHHHH | 23.24 | - | |
| 289 | N-linked_Glycosylation | LAEKDAVNESGRVEF HHHHHCCCCCCCCCC | 39.16 | UniProtKB CARBOHYD | |
| 402 | Phosphorylation | YIRKAPRSHTLLSPS EEEECCCCCCCCCCC | 21.55 | 22210691 | |
| 404 | Phosphorylation | RKAPRSHTLLSPSPK EECCCCCCCCCCCCC | 30.55 | 29978859 | |
| 407 | Phosphorylation | PRSHTLLSPSPKPKK CCCCCCCCCCCCCCC | 26.83 | 28450419 | |
| 409 | Phosphorylation | SHTLLSPSPKPKKSV CCCCCCCCCCCCCEE | 42.03 | 28450419 | |
| 424 | Ubiquitination | VVKKKKFKLDKDNGV EEEECEEECCCCCCC | 65.73 | - | |
| 439 | Phosphorylation | TPGEKMLTVPHITCD CCCCCEEECCEEECC | 28.61 | 24043423 | |
| 444 | Phosphorylation | MLTVPHITCDPPEER EEECCEEECCCCHHH | 14.07 | 24043423 | |
| 445 | S-nitrosylation | LTVPHITCDPPEERR EECCEEECCCCHHHC | 7.96 | 22178444 | |
| 445 | S-nitrosocysteine | LTVPHITCDPPEERR EECCEEECCCCHHHC | 7.96 | - | |
| 457 | Phosphorylation | ERRLDHFSVDGYDSS HHCCCCCCCCCCCCC | 18.84 | 28348404 | |
| 461 | Phosphorylation | DHFSVDGYDSSVRKS CCCCCCCCCCCCCCC | 14.27 | 24043423 | |
| 463 | Phosphorylation | FSVDGYDSSVRKSPT CCCCCCCCCCCCCCC | 23.42 | 24043423 | |
| 464 | Phosphorylation | SVDGYDSSVRKSPTL CCCCCCCCCCCCCCE | 24.04 | 22817900 | |
| 468 | Phosphorylation | YDSSVRKSPTLLEVS CCCCCCCCCCEEEEE | 16.74 | 28857561 | |
| 470 | Phosphorylation | SSVRKSPTLLEVSMP CCCCCCCCEEEEECC | 51.58 | 22817900 | |
| 475 | Phosphorylation | SPTLLEVSMPHFMRT CCCEEEEECCCCCCC | 19.74 | 23312004 | |
| 482 | Phosphorylation | SMPHFMRTNSFAEDL ECCCCCCCCCCCHHC | 24.35 | 28450419 | |
| 484 | Phosphorylation | PHFMRTNSFAEDLDL CCCCCCCCCCHHCCC | 25.81 | 26657352 | |
| 513 | Phosphorylation | LREHHRATIKVIRRM HHHHHHHHHHHHHHH | 22.68 | 22817900 | |
| 577 | Phosphorylation | PSLFISVSEKSKDRG CEEEEEECHHHCCCC | 32.30 | 22817900 | |
| 666 | Phosphorylation | LPTYEQLTVPRRGPD CCCCCCCCCCCCCCC | 28.32 | 24719451 |
| Modified Location | Modified Residue | Modification | Function | Reference |
|---|---|---|---|---|
| 27 | S | Phosphorylation |
| 11799244 |
* 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 !! | ||||||
| Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
|---|---|---|---|---|
| AKAP9_HUMAN | AKAP9 | physical | 11799244 | |
| KAP2_HUMAN | PRKAR2A | physical | 11799244 | |
| PP1A_HUMAN | PPP1CA | physical | 11799244 | |
| KAPCA_HUMAN | PRKACA | physical | 11799244 | |
| KCNE1_HUMAN | KCNE1 | physical | 11101505 | |
| KCNE2_HUMAN | KCNE2 | physical | 11101505 | |
| KCNQ2_HUMAN | KCNQ2 | physical | 12524525 | |
| HSP74_HUMAN | HSPA4 | physical | 19114714 | |
| KCNE1_HUMAN | KCNE1 | physical | 19114714 | |
| NEDD4_HUMAN | NEDD4 | physical | 22024150 | |
| UBP2_HUMAN | USP2 | physical | 22024150 | |
| AP2M1_HUMAN | AP2M1 | physical | 23529131 | |
| NED4L_HUMAN | NEDD4L | physical | 23529131 | |
| NED4L_HUMAN | NEDD4L | physical | 26405101 | |
| BACE1_HUMAN | BACE1 | physical | 26454161 | |
| AKAP9_HUMAN | AKAP9 | physical | 18093912 | |
| KCNE1_HUMAN | KCNE1 | physical | 24713462 | |
| AKAP9_HUMAN | AKAP9 | physical | 24713462 |
| 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 | |
| 192500 | Long QT syndrome 1 (LQT1) |
| 220400 | Jervell and Lange-Nielsen syndrome 1 (JLNS1) |
| 607554 | Atrial fibrillation, familial, 3 (ATFB3) |
| 609621 | Short QT syndrome 2 (SQT2) |
| 125853 | Diabetes 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 | |
| DB01244 | Bepridil |
| DB00808 | Indapamide |
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