Names & Taxonomy

Uniprot ID:
P51787
Entry Name:
KCNQ1_HUMAN
Status:
reviewed
Protein Names:
Potassium voltage-gated channel subfamily KQT member 1 (IKs producing slow voltage-gated potassium channel subunit alpha KvLQT1) (KQT-like 1) (Voltage-gated potassium channel subunit Kv7.1)
Gene Names:
KCNQ1 KCNA8 KCNA9 KVLQT1
Gene Names Primary:
KCNQ1
Organism:
Homo sapiens (Human)

Structure

Length:
676
Sequence:
MAAASSPPRAERKRWGWGRLPGARRGSAGLAKKCPFSLELAEGGPAGGALYAPIAPGAPGPAPPASPAAPAAPPVASDLGPRPPVSLDPRVSIYSTRRPVLARTHVQGRVYNFLERPTGWKCFVYHFAVFLIVLVCLIFSVLSTIEQYAALATGTLFWMEIVLVVFFGTEYVVRLWSAGCRSKYVGLWGRLRFARKPISIIDLIVVVASMVVLCVGSKGQVFATSAIRGIRFLQILRMLHVDRQGGTWRLLGSVVFIHRQELITTLYIGFLGLIFSSYFVYLAEKDAVNESGRVEFGSYADALWWGVVTVTTIGYGDKVPQTWVGKTIASCFSVFAISFFALPAGILGSGFALKVQQKQRQKHFNRQIPAAASLIQTAWRCYAAENPDSSTWKIYIRKAPRSHTLLSPSPKPKKSVVVKKKKFKLDKDNGVTPGEKMLTVPHITCDPPEERRLDHFSVDGYDSSVRKSPTLLEVSMPHFMRTNSFAEDLDLEGETLLTPITHISQLREHHRATIKVIRRMQYFVAKKKFQQARKPYDVRDVIEQYSQGHLNLMVRIKELQRRLDQSIGKPSLFISVSEKSKDRGSNTIGARLNRVEDKVTQLDQRLALITDMLHQLLSLHGGSTPGSGGPPREGGAHITQPCGSGGSVDPELFLPSNTLPTYEQLTVPRRGPDEGS
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cell membrane
Intramembrane:
INTRAMEM 300 320 Pore-forming; Name=Segment H5.

Function

Function:
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) (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505, PubMed:19687231). Induces a voltage-dependent by rapidly activating and slowly deactivating potassium-selective outward current (By similarity) (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505). Promotes also a delayed voltage activated potassium current showing outward rectification characteristic (By similarity). During beta-adrenergic receptor stimulation participates in cardiac repolarization by associating with KCNE1 to form the I(Ks) cardiac potassium current that increases the amplitude and slows down the activation kinetics of outward potassium current I(Ks) (By similarity) (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505). Muscarinic agonist oxotremorine-M strongly suppresses KCNQ1/KCNE1 current (PubMed:10713961). When associated with KCNE3, forms the potassium channel that is important for cyclic AMP-stimulated intestinal secretion of chloride ions (PubMed:10646604). This interaction with KCNE3 is reduced by 17beta-estradiol, resulting in the reduction of currents (By similarity). During conditions of increased substrate load, maintains the driving force for proximal tubular and intestinal sodium ions absorption, gastric acid secretion, and cAMP-induced jejunal chloride ions secretion (By similarity). Allows the provision of potassium ions to the luminal membrane of the secretory canaliculus in the resting state as well as during stimulated acid secretion (By similarity). When associated with KCNE2, forms a heterooligomer complex leading to currents with an apparently instantaneous activation, a rapid deactivation process and a linear current-voltage relationship and decreases the amplitude of the outward current (PubMed:11101505). When associated with KCNE4, inhibits voltage-gated potassium channel activity (PubMed:19687231). When associated with KCNE5, this complex only conducts current upon strong and continued depolarization (PubMed:12324418). Also forms a heterotetramer with KCNQ5; has a voltage-gated potassium channel activity (PubMed:24855057). Binds with phosphatidylinositol 4,5-bisphosphate (PubMed:25037568).
Cross Reference Drug Bank:
DB01244 DB00808
Gene Ontology Go:
basolateral plasma membrane
cytoplasm
cytoplasmic vesicle membrane
early endosome
endoplasmic reticulum
ion channel complex
late endosome
lysosome
membrane raft
plasma membrane
voltage-gated potassium channel complex
calmodulin binding
delayed rectifier potassium channel activity
ion channel binding
outward rectifier potassium channel activity
phosphatidylinositol-4,5-bisphosphate binding
protein kinase A catalytic subunit binding
protein kinase A regulatory subunit binding
protein phosphatase 1 binding
scaffold protein binding
voltage-gated potassium channel activity
voltage-gated potassium channel activity involved in atrial cardiac muscle cell action potential repolarization
voltage-gated potassium channel activity involved in cardiac muscle cell action potential repolarization
voltage-gated potassium channel activity involved in ventricular cardiac muscle cell action potential repolarization
atrial cardiac muscle cell action potential
cardiac conduction
cardiac muscle contraction
cardiovascular system development
cellular response to cAMP
cellular response to drug
cellular response to epinephrine stimulus
gene silencing
inner ear development
intestinal absorption
membrane repolarization during action potential
membrane repolarization during cardiac muscle cell action potential
negative regulation of delayed rectifier potassium channel activity
negative regulation of voltage-gated potassium channel activity
positive regulation of cardiac muscle contraction
positive regulation of defense response to virus by host
positive regulation of heart rate
positive regulation of potassium ion transmembrane transport
potassium ion export
potassium ion export across plasma membrane
potassium ion transmembrane transport
regulation of atrial cardiac muscle cell membrane repolarization
regulation of gastric acid secretion
regulation of gene expression by genetic imprinting
regulation of heart contraction
regulation of heart rate by cardiac conduction
regulation of membrane repolarization
regulation of ventricular cardiac muscle cell membrane repolarization
renal absorption
sensory perception of sound
synaptic transmission
ventricular cardiac muscle cell action potential
xenophagy
Gene Ontology Biological Process:
atrial cardiac muscle cell action potential
cardiac conduction
cardiac muscle contraction
cardiovascular system development
cellular response to cAMP
cellular response to drug
cellular response to epinephrine stimulus
gene silencing
inner ear development
intestinal absorption
membrane repolarization during action potential
membrane repolarization during cardiac muscle cell action potential
negative regulation of delayed rectifier potassium channel activity
negative regulation of voltage-gated potassium channel activity
positive regulation of cardiac muscle contraction
positive regulation of defense response to virus by host
positive regulation of heart rate
positive regulation of potassium ion transmembrane transport
potassium ion export
potassium ion export across plasma membrane
potassium ion transmembrane transport
regulation of atrial cardiac muscle cell membrane repolarization
regulation of gastric acid secretion
regulation of gene expression by genetic imprinting
regulation of heart contraction
regulation of heart rate by cardiac conduction
regulation of membrane repolarization
regulation of ventricular cardiac muscle cell membrane repolarization
renal absorption
sensory perception of sound
synaptic transmission
ventricular cardiac muscle cell action potential
xenophagy
Gene Ontology Molecular Function:
calmodulin binding
delayed rectifier potassium channel activity
ion channel binding
outward rectifier potassium channel activity
phosphatidylinositol-4,5-bisphosphate binding
protein kinase A catalytic subunit binding
protein kinase A regulatory subunit binding
protein phosphatase 1 binding
scaffold protein binding
voltage-gated potassium channel activity
voltage-gated potassium channel activity involved in atrial cardiac muscle cell action potential repolarization
voltage-gated potassium channel activity involved in cardiac muscle cell action potential repolarization
voltage-gated potassium channel activity involved in ventricular cardiac muscle cell action potential repolarization
Gene Ontology Cellular Component:
basolateral plasma membrane
cytoplasm
cytoplasmic vesicle membrane
early endosome
endoplasmic reticulum
ion channel complex
late endosome
lysosome
membrane raft
plasma membrane
voltage-gated potassium channel complex
Keywords:
3D-structure
Alternative splicing
Atrial fibrillation
Calmodulin-binding
Cell membrane
Coiled coil
Complete proteome
Cytoplasmic vesicle
Deafness
Diabetes mellitus
Disease mutation
Endoplasmic reticulum
Endosome
Glycoprotein
Ion channel
Ion transport
Long QT syndrome
Membrane
Phosphoprotein
Polymorphism
Potassium
Potassium channel
Potassium transport
Reference proteome
Short QT syndrome
Transmembrane
Transmembrane helix
Transport
Ubl conjugation
Voltage-gated channel
Interacts With:
P15382

Publication

PubMed ID:
9312006 9305853 9799083 10024302 14702039 16554811 15489334 9108097 8900283 8528244 10654932 11101505 10713961 10646604 12324418 11799244 19687231 20533308 21228319 22024150 23529131 24855057 18165683 19693805 9323054 10704188 8872472 8818942 9024139 9386136 9272155 9302275 9570196 9641694 9781056 9693036 9482580 9702906 10090886 10367071 9927399 10482963 10220144 10220146 10409658 10728423 10973849 12442276 12522251 15159330 15840476 16414944 16922724 18400097 18441444 18711367 18711366 24390345 19716085 19540844 21241800 25139741 24269949 24184248 25037568 24713462 25705178