Page last updated: 2024-09-27

Long QT Syndrome 1

A form of long QT syndrome that is without congenital deafness. It is caused by mutation of the KCNQ1 gene which encodes a protein in the VOLTAGE-GATED POTASSIUM CHANNEL.

Synonyms(5)

Synonym
Ward-Romano Syndrome
Ventricular Fibrillation with Prolonged QT Interval
Long QT Syndrome 1
Long QT Syndrome Type 1
Romano-Ward Syndrome

Research

Studies (35)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's8 (22.86)18.1394
2010's23 (65.71)28.8240
2020's4 (11.43)9.53
DrugIndicatedRelationship StrengthStudiesTrials
urea0low10
bisoprolol0low10
bupivacaine0low10
citalopram0low10
e 40310low10
isoproterenol0low20
propofol0low10
propranolol0medium11
sevoflurane0low10
terfenadine0low10
alanine0low20
aspartic acid0low10
thiazoles0low10
sq-117250low10
nicorandil0low10
fomesafen0medium11
terlipressin0low10
landiolol0low10
glycerophosphoinositol 4,5-bisphosphate0low10
proline0low20
lypressin0low10
phenylephrine hydrochloride0low10
piperidines0low20
(r)-n-(4-(4-methoxyphenyl)thiazol-2-yl)-1-tosylpiperidine-2-carboxamide0low10
guanosine triphosphate0low10

Protein Targets (360)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
cytochrome P450 family 3 subfamily A polypeptide 45005
G2002
potassium voltage-gated channel subfamily KQT member 1 isoform 10011
Potassium voltage-gated channel subfamily KQT member 20011
Interferon beta3003
HLA class I histocompatibility antigen, B alpha chain 2002
Potassium voltage-gated channel subfamily KQT member 10011
Potassium voltage-gated channel subfamily KQT member 40011
Potassium voltage-gated channel subfamily H member 20718
Inositol hexakisphosphate kinase 12002
cytochrome P450 2C9, partial2002
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE3003
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
Carbonic anhydrase 10103
Carbonic anhydrase 20103
Alanine racemase, biosynthetic0001
5-hydroxytryptamine receptor 1D0002
Glutamate receptor ionotropic, NMDA 1 0314
Proton-coupled amino acid transporter 10202
Adenosine deaminase0001
Carbonic anhydrase 40002
Chain A, Ferritin light chain3003
phosphopantetheinyl transferase4004
aldehyde dehydrogenase 1 family, member A14004
arylsulfatase A4004
euchromatic histone-lysine N-methyltransferase 28008
Bloom syndrome protein isoform 14004
cytochrome P450 2C19 precursor3003
lethal factor (plasmid)4004
lamin isoform A-delta106006
Metabotropic glutamate receptor 60011
Excitatory amino acid transporter 40101
Glutamate transporter homolog0011
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase0101
Excitatory amino acid transporter 10101
Excitatory amino acid transporter 20101
Excitatory amino acid transporter 30101
Glutamate receptor ionotropic, NMDA 2A 0112
Glutamate receptor ionotropic, NMDA 2B0112
Glutamate receptor ionotropic, NMDA 2C0112
Metabotropic glutamate receptor 10011
Metabotropic glutamate receptor 20011
Carbonic anhydrase-like protein, putative0001
Glutamate receptor ionotropic, NMDA 2D0112
Glutamate receptor ionotropic, NMDA 3B0112
Glutamate receptor ionotropic, NMDA 3A0112
ATAD5 protein, partial3003
chromobox protein homolog 16006
DNA polymerase kappa isoform 12002
Beta-2 adrenergic receptor05511
Beta-1 adrenergic receptor0448
Beta-3 adrenergic receptor0347
Beta-1 adrenergic receptor0314
Potassium channel subfamily K member 30101
Potassium channel subfamily K member 30101
Potassium channel subfamily K member 20011
Potassium channel subfamily K member 90101
thioredoxin reductase4004
EWS/FLI fusion protein3003
NPC intracellular cholesterol transporter 1 precursor2002
D(1A) dopamine receptor4004
survival motor neuron protein isoform d1001
Solute carrier family 22 member 1 0202
ATP-binding cassette sub-family C member 30404
Multidrug resistance-associated protein 40404
Sodium-dependent noradrenaline transporter0101
5-hydroxytryptamine receptor 40808
Bile salt export pump0505
Aldo-keto reductase family 1 member B10606
Tryptophan 5-hydroxylase 10101
Angiotensin-converting enzyme0202
Aldo-keto reductase family 1 member B10101
5-hydroxytryptamine receptor 1A0315
Acetylcholinesterase0101
Aromatase0101
Sodium-dependent noradrenaline transporter 0606
Sodium-dependent dopamine transporter0202
Alpha-1D adrenergic receptor0326
5-hydroxytryptamine receptor 2C0819
Sodium-dependent serotonin transporter0415
Sodium-dependent serotonin transporter0101
Histamine H1 receptor0404
5-hydroxytryptamine receptor 2B0819
5-hydroxytryptamine receptor 70202
Spike glycoprotein5005
Sodium-dependent dopamine transporter 0303
Sigma intracellular receptor 20101
Sodium-dependent serotonin transporter0101
Sodium-dependent dopamine transporter0101
Nuclear receptor subfamily 3 group C member 3 0202
Canalicular multispecific organic anion transporter 10404
Ataxin-23003
Sigma non-opioid intracellular receptor 10404
Sigma non-opioid intracellular receptor 10101
Transporter0202
ATP-dependent phosphofructokinase2002
regulator of G-protein signaling 42002
cytochrome P450 2D6 isoform 11001
cytochrome P450 3A4 isoform 12002
histone acetyltransferase KAT2A isoform 11001
Gamma-aminobutyric acid receptor subunit pi2226
Gamma-aminobutyric acid receptor subunit beta-12226
Gamma-aminobutyric acid receptor subunit delta2226
Gamma-aminobutyric acid receptor subunit gamma-22226
Gamma-aminobutyric acid receptor subunit alpha-52226
Gamma-aminobutyric acid receptor subunit alpha-32226
Gamma-aminobutyric acid receptor subunit gamma-12226
Gamma-aminobutyric acid receptor subunit alpha-22226
Gamma-aminobutyric acid receptor subunit alpha-42226
Gamma-aminobutyric acid receptor subunit gamma-32226
Gamma-aminobutyric acid receptor subunit alpha-62226
Gamma-aminobutyric acid receptor subunit alpha-12226
Gamma-aminobutyric acid receptor subunit beta-32226
Gamma-aminobutyric acid receptor subunit beta-22226
GABA theta subunit2226
Gamma-aminobutyric acid receptor subunit epsilon2226
Luciferase1001
hypoxia-inducible factor 1 alpha subunit2002
progesterone receptor2002
estrogen-related nuclear receptor alpha6006
pregnane X nuclear receptor3003
estrogen nuclear receptor alpha5005
peroxisome proliferator activated receptor gamma4004
vitamin D (1,25- dihydroxyvitamin D3) receptor1001
Histone H2A.x5005
nuclear factor erythroid 2-related factor 2 isoform 15005
Chain A, Uracil Phosphoribosyltransferase0011
Chain B, Uracil Phosphoribosyltransferase0011
Chain C, Uracil Phosphoribosyltransferase0011
TDP1 protein5005
67.9K protein2002
2-dehydropantoate 2-reductase0011
Ras-related protein Rab-7a0011
4-galactosyl-N-acetylglucosaminide 3-alpha-L-fucosyltransferase 90202
glp-1 receptor, partial2002
GLS protein3003
GLI family zinc finger 36006
Microtubule-associated protein tau2002
nuclear receptor subfamily 1, group I, member 33003
NPYLR7B0011
cytochrome P450 2D62002
Beta-1 adrenergic receptor 0125
thyroid stimulating hormone receptor3003
activating transcription factor 64004
beta-2 adrenergic receptor3003
hexokinase-4 isoform 11001
vitamin D3 receptor isoform VDRA1001
glucokinase regulatory protein1001
serine/threonine-protein kinase mTOR isoform 12002
geminin4004
peripheral myelin protein 224004
Bile salt export pump0202
Beta-2 adrenergic receptor0416
Polyunsaturated fatty acid lipoxygenase ALOX150202
D(2) dopamine receptor0415
Alpha-1B adrenergic receptor0415
Beta-1 adrenergic receptor0428
Beta-2 adrenergic receptor0011
Alpha-2B adrenergic receptor0314
D(1A) dopamine receptor0415
D(4) dopamine receptor0101
Alpha-2C adrenergic receptor0314
Alpha-2A adrenergic receptor0314
Alpha-1D adrenergic receptor0415
Beta-adrenergic receptor kinase 10011
Beta-3 adrenergic receptor0011
Beta-3 adrenergic receptor0314
Vasopressin V1a receptor0011
Alpha-1A adrenergic receptor0236
Alpha-1B adrenergic receptor0124
D(3) dopamine receptor0303
Alpha-1A adrenergic receptor0415
Histamine H2 receptor1012
D(1A) dopamine receptor2002
Beta-2 adrenergic receptor0202
Taste receptor type 2 member 380011
Taste receptor type 2 member 390011
Taste receptor type 2 member 400011
Taste receptor type 2 member 410011
Taste receptor type 2 member 430011
Taste receptor type 2 member 310011
Taste receptor type 2 member 450011
Taste receptor type 2 member 460011
Taste receptor type 2 member 300011
Taste receptor type 2 member 190011
Taste receptor type 2 member 200011
Taste receptor type 2 member 500011
Taste receptor type 2 member 600011
Oxytocin receptor0011
Beta-2 adrenergic receptor 0112
Taste receptor type 2 member 420011
Beta-2 adrenergic receptor0135
Taste receptor type 2 member 160011
Taste receptor type 2 member 140011
Taste receptor type 2 member 130011
Taste receptor type 2 member 100011
Taste receptor type 2 member 90011
Taste receptor type 2 member 80011
Taste receptor type 2 member 70011
Taste receptor type 2 member 50011
Taste receptor type 2 member 40011
Taste receptor type 2 member 30011
Taste receptor type 2 member 10011
2,3-bisphosphoglycerate-independent phosphoglycerate mutase1001
Cellular tumor antigen p534004
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
15-lipoxygenase, partial1001
RAR-related orphan receptor gamma4004
SMAD family member 22002
SMAD family member 32002
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a3003
v-jun sarcoma virus 17 oncogene homolog (avian)3003
cytochrome P450 2C9 precursor1001
Chain A, 2-oxoglutarate Oxygenase1001
GALC protein1001
AR protein3003
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)2002
glucocorticoid receptor [Homo sapiens]2002
IDH12002
lysosomal alpha-glucosidase preproprotein2002
mu-type opioid receptor isoform MOR-10011
Vpr1001
5-hydroxytryptamine receptor 2A0011
Neuronal acetylcholine receptor subunit alpha-42002
Neuronal acetylcholine receptor subunit beta-22002
Glutamate 5-kinase0001
Angiotensin-converting enzyme 0101
Neutral amino acid transporter A0101
Neutral amino acid transporter B(0)0101
Amino acid transporter0101
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
Chain A, Ferritin light chain0011
USP1 protein, partial2002
caspase 7, apoptosis-related cysteine protease3003
thyroid stimulating hormone receptor1001
retinoic acid nuclear receptor alpha variant 13003
farnesoid X nuclear receptor2002
caspase-33003
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a2002
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 11001
thyroid hormone receptor beta isoform 22002
heat shock protein beta-12002
DNA polymerase iota isoform a (long)4004
muscarinic acetylcholine receptor M12002
Fatty-acid amide hydrolase 10101
UDP-glucuronosyltransferase 1A90001
Gamma-aminobutyric acid receptor subunit alpha-10001
Gamma-aminobutyric acid receptor subunit beta-10001
Gamma-aminobutyric acid receptor subunit alpha-20001
Gamma-aminobutyric acid receptor subunit alpha-30001
Gamma-aminobutyric acid receptor subunit alpha-10112
Gamma-aminobutyric acid receptor subunit beta-10011
Gamma-aminobutyric acid receptor subunit gamma-20112
Gamma-aminobutyric acid receptor subunit alpha-40001
Gamma-aminobutyric acid receptor subunit gamma-20001
Prostaglandin G/H synthase 10101
Sodium- and chloride-dependent GABA transporter 10101
Gamma-aminobutyric acid receptor subunit beta-30112
Gamma-aminobutyric acid receptor subunit alpha-50011
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Gamma-aminobutyric acid receptor subunit alpha-30011
Gamma-aminobutyric acid receptor subunit alpha-20011
Gamma-aminobutyric acid receptor subunit beta-20011
Sodium- and chloride-dependent betaine transporter0101
Gamma-aminobutyric acid receptor subunit alpha-40011
Fatty-acid amide hydrolase 10101
Gamma-aminobutyric acid receptor subunit alpha-60011
UDP-glucuronosyltransferase 1A80001
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Albumin0011
Cytochrome P450 1A20101
Muscarinic acetylcholine receptor M20112
ATP-dependent translocase ABCB10303
Cytochrome P450 3A40202
5-hydroxytryptamine receptor 1A0101
Alpha-2A adrenergic receptor0124
Adenosine receptor A30112
Cytochrome P450 2C80101
Cytochrome P450 2D60101
Muscarinic acetylcholine receptor M10213
Cytochrome P450 2C9 0101
Alpha-2B adrenergic receptor0012
Alpha-2C adrenergic receptor0326
D(3) dopamine receptor0101
Muscarinic acetylcholine receptor M30112
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Endothelin-1 receptor0112
5-hydroxytryptamine receptor 1B0213
5-hydroxytryptamine receptor 2A0415
5-hydroxytryptamine receptor 1B0202
Adenosine receptor A2a0112
Neuromedin-K receptor0011
Type-1 angiotensin II receptor0112
Cholecystokinin receptor type A0112
Cytochrome P450 2C190101
5-hydroxytryptamine receptor 70112
Mu-type opioid receptor0112
Vasopressin V1a receptor0112
Delta-type opioid receptor0112
Kappa-type opioid receptor0112
5-hydroxytryptamine receptor 60415
Cytochrome P450 2J20202
Voltage-dependent L-type calcium channel subunit alpha-1C0202
Sodium channel protein type 5 subunit alpha0202
Sodium/bile acid cotransporter0101
Nuclear receptor subfamily 2 group E member 10022
Chain A, ATP-DEPENDENT DNA HELICASE Q11001
transcriptional regulator ERG isoform 31001
Fatty acid-binding protein, liver0101
endonuclease IV1001
Thrombopoietin1001
estrogen receptor 2 (ER beta)1001
retinoid X nuclear receptor alpha2002
glucocerebrosidase1001
Parkin1001
peroxisome proliferator-activated receptor delta2002
XBP10101
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)1001
Caspase-71001
peripheral myelin protein 22 isoform 11001
atrial natriuretic peptide receptor 1 precursor1001
potassium voltage-gated channel subfamily H member 2 isoform d1001
caspase-31001
histone deacetylase 9 isoform 31001
huntingtin isoform 21001
ubiquitin-conjugating enzyme E2 N0101
ras-related protein Rab-9A1001
DNA polymerase eta isoform 11001
DNA damage-inducible transcript 3 protein0101
nuclear receptor ROR-gamma isoform 11001
M-phase phosphoprotein 81001
neuropeptide S receptor isoform A1001
Voltage-dependent L-type calcium channel subunit alpha-1F0101
Voltage-dependent calcium channel gamma-2 subunit1001
Epidermal growth factor receptor0101
Tyrosine-protein kinase Fyn0101
ATP-dependent translocase ABCB10101
DNA gyrase subunit B0101
Transcriptional activator protein LuxR0101
Dipeptidyl peptidase 40101
D0101
Glutamate receptor 21001
DNA gyrase subunit A0101
ATP-dependent translocase ABCB10101
Substance-K receptor0202
Histamine H1 receptor0101
C-C chemokine receptor type 50202
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Rap guanine nucleotide exchange factor 31001
Urease subunit alpha0101
Urease subunit beta0101
Olfactory receptor 51E20011
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]