Long Qt Syndrome malady

Disease Ontology: An autosomal genetic disease that is characterized by delayed repolarization of the heart following a heartbeat increases the risk of episodes of torsade de pointes (tdp, a form of irregular heartbeat that originates from the ventricles).⁶

MalaCards: Long Qt Syndrome, also known as romano-ward syndrome, is related to long qt syndrome 1 and long qt syndrome 2. An important gene associated with Long Qt Syndrome is SCN5A (sodium channel, voltage-gated, type V, alpha subunit), and among its related pathways are Potassium transporters- inward current and Adrenoceptors. The compounds chromanol and flecainide have been mentioned in the context of this disorder. Affiliated tissues include brain, heart and t cells, and related mouse phenotypes are no phenotypic analysis and muscle.

Wikipedia: The long QT syndrome (LQTS) is a rare inborn heart condition in which delayed repolarization of the...⁴⁴ more...

Aliases & Descriptions:

long qt syndrome 6 7 17 8 32 43 romano-ward syndrome 6 15 43 long qt syndrome, autosomal dominant 15 romano-ward syndrome (disorder) 6

romano-ward long qt syndrome 15 long q-t syndrome 6 lqt 6

Disease types for long qt syndrome family:

long qt syndrome 1	long qt syndrome 10
long qt syndrome 11	long qt syndrome 2
long qt syndrome 3	long qt syndrome 4
long qt syndrome 5	long qt syndrome 6
long qt syndrome 8	long qt syndrome 9
long qt syndrome 12	long qt syndrome 13

Diseases related to long qt syndrome by text searches and GeneDecks gene sharing:

(show top 50)    (show all 100)

id	Related Disease	Score	Top Affiliating Genes
1	long qt syndrome 1	35.1	SCN5A, KCNE2, KCNQ1, KCNH2, KCNE1
2	long qt syndrome 2	34.7	KCNH2, SCN5A
3	jervell-lange nielsen syndrome	34.2	KCNQ1, KCNE1
4	brugada syndrome	32.0	RYR2, NOS1AP, ANK2, FKBP1B, SCN5A, KCNJ5
5	familial atrial fibrillation	31.8	KCNE2, KCNQ1, SCN5A
6	sinusitis	31.4	CAV3, ZACN, HCN4, ADRB1, ANK2, SCN5A
7	sudden infant death syndrome	31.4	KCND3, KCNJ2, KCNH2, KCNE1, KCNQ1, KCNE2
8	conduction disease	30.0	SCN4B, SCN1B, SCN5A
9	fainting	29.5	HCN4, SCN5A, SCN1B, KCNH2, ADRB1, KCNE3
10	conduct disorder	29.2	SCN5A, NOS1, ADRA2C, KCNH2, KCNQ1
11	sudden cardiac death multi-gene panels	29.1	FKBP1B, SCN5A, KCNE2, KCNQ1, KCNJ2, KCNH2
12	twinning	28.6	SCN5A, SHH, KCNQ1OT1, KCNE2, ADRB1, KCNH2
13	ventricular fibrillation	28.5	KCNJ5, KCNE2, KCNQ1, KCNJ2, KCNH2, KCNE3
14	congenital heart defect	28.4	MYH7, ADRB1, ADRA2C, ADRB2, RYR2, SLN
15	aldosteronism	28.3	KCNE1, ADRA2C, ADRB1, MYH7, KCNQ1, KCNJ5
16	syncope	27.7	KCNH2, KCNJ2, KCNQ1, KCNE2, KCNJ5, SCN4B
17	seizures	27.6	KCND2, KCNH2, KCNQ1, KCNE2, KCNQ2, NOS1
18	atrial fibrillation	27.6	KCNQ1, HCN4, SLN, RYR2, ADRB2, ADRB1
19	cystic fibrosis	23.6	ADRB1, KCNQ1, ATF6, CANX, AKAP13, NOS1
20	sinus bradycardia syndrome	13.7	KCNH2, ANK2, KCNQ1
21	short qt syndrome	13.7	KCNH2, KCNJ2, KCNQ1
22	heart block	13.6	SCN5A, KCNE2, KCNE1, KCNQ1
23	catecholaminergic polymorphic ventricular tachycardia	13.6	SCN5A, KCNH2
24	alpha 1-antitrypsin deficiency	13.6	MYH7, KCNH2, SCN5A, RYR2
25	congenital heart block	13.6	KCNE1, KCNQ1, KCNE2, SCN4B, SCN5A
26	hypokalemic periodic paralysis	13.5	KCNE3, KCNE1L, KCNJ2
27	congestive heart failure and beta-blocker response, modifier of	13.5	ADRB1, ADRA2C
28	hyperkalemic periodic paralysis	13.5	KCNJ2, SCN1B, KCNE3
29	left ventricular noncompaction	13.5	SNTA1, MYH7, FKBP1B, RYR2, SCN5A
30	sick sinus syndrome	13.4	HCN4, ANK2, SCN5A
31	familial hypertrophic cardiomyopathy	13.4	MYH7, CAV3, FKBP1B, SLN
32	right bundle branch block	13.3	SCN5A, HCN4, SCN1B, KCNE3
33	arrhythmogenic right ventricular dysplasia	13.3	SCN5A, FKBP1B, RYR2
34	periodic paralyses	13.3	KCNJ2, KCNE3
35	resting heart rate	13.2	ADRB1, ADRB2, MYH7, SCN5A
36	orthostatic intolerance	13.2	ADRA2C, ADRB1, ADRB2
37	hypokalemia	13.1	KCNJ5, KCNQ1, KCNH2, KCNE3, KCNE1, ADRB2
38	benign neonatal seizures	13.1	KCNQ2, KCNQ3
39	myokymia with neonatal epilepsy	13.1	KCNQ3, KCNQ2
40	congenital aortic valve stenosis	13.1	ADRB2, ADRB1
41	hypertrophic cardiomyopathy	13.0	CAV3, HCN4, SLN, RYR2, MYH7, FKBP1B
42	gastroparesis	13.0	KCNE2, NOS1, KCNQ1, KCNE1, KCNH2
43	benign familial neonatal-infantile seizures	13.0	SCN1B, KCNQ3, KCNQ2
44	autosomal dominant nocturnal frontal lobe epilepsy	13.0	KCNQ2, KCNQ3, SCN1B
45	episodic ataxia	13.0	KCNQ3, KCND3, KCNH2
46	febrile seizures	13.0	KCNQ3, SCN1B, KCNQ2
47	generalized epilepsy with febrile seizures plus	13.0	KCNQ3, KCNQ2, SCN1B
48	myokymia	12.9	KCNA5, KCND3, KCNQ2, KCNQ3
49	idiopathic generalized epilepsy	12.8	KCNQ2, KCNQ3, ZACN, SCN1B
50	paralysis	12.8	KCNJ2, KCNJ5, SCN8A, ADRB2, KCNE3

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to long qt syndrome:

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MGI Mouse Phenotypes related to long qt syndrome:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	no phenotypic analysis	MP:0003012	7.4	STK11, RYR2, ADRB2, ADRB1, MYH7, HCN4
2	muscle phenotype	MP:0005369	7.1	SHH, HCN4, STK11, SLN, RYR2, ADRB2
3	nervous system phenotype	MP:0003631	6.8	SNTA1, NOS1, SHH, STK11, RYR2, ADRA2C
4	cardiovascular system phenotype	MP:0005385	6.6	KCNA5, NOS1, SHH, HCN4, STK11, SLN
5	growth/size phenotype	MP:0005378	6.2	AKAP13, PTRF, NOS1, SHH, STK11, RYR2
6	homeostasis/metabolism phenotype	MP:0005376	6.2	AKAP13, PTRF, SNTA1, NOS1, SHH, STK11
7	behavior/neurological phenotype	MP:0005386	6.0	PTRF, SNTA1, NOS1, SHH, STK11, RYR2
8	mortality/aging	MP:0010768	5.4	CANX, AKAP13, NOS1, SHH, HCN4, STK11

Articles related to long qt syndrome:

(show top 50)    (show all 342)

id	Title	Authors	Year	Affiliating Genes
1	Trafficking defect and proteasomal degradation contri bute to the phenotype of a novel KCNH2 long QT syndrome mutation. (21483829)	Mihic A.... Tsushima R.G.	2011	KCNH2
2	Biophysical characterization of KCNQ1 P320 mutations linked to long QT syndrome 1. (19540844)	Thomas D.... Zehelein J.	2010	KCNQ1
3	The response of the QT interval to the brief tachycar dia provoked by standing: a bedside test for diagnosing long QT syndrome. (20116193)	Viskin S.... Wilde A.A.	2010	KCNH2, SCN5A, KCNH1
4	Mutation-specific risk in two genetic forms of type 3 long QT syndrome. (20102920)	Liu J.F.... Zareba W.	2010	SCN5A
5	Long QT syndrome with compound mutations is associate d with a more severe phenotype: a Japanese multicenter study. (20541041)	Itoh H.... Horie M.	2010	KCNQ1, KCNH2
6	Utility of the recovery electrocardiogram after exerc ise: a novel indicator for the diagnosis and genotyping of long QT syndrome? (20226272)	Chattha I.S.... Krahn A.D.	2010	KCNQ1, KCNH2
7	Trafficking-deficient long QT syndrome mutation KCNQ1 -T587M confers severe clinical phenotype by impairment of KCNH2 membrane locali zation: evidence for clinically significant IKr-IKs alpha-subunit interaction. (19959132)	Biliczki P.... Ehrlich J.R.	2009	KCNQ1, KCNH2
8	Genetic in long QT syndromes (20361479)	Iturralde-Torres P.... Medeiros-Domingo A.	2009	KCNE2
9	Contribution of long-QT syndrome genetic variants in sudden infant death syndrome. (19322600)	Millat G.... Rousson R.	2009	KCNQ1, KCNH2, KCNE1
10	Long QT syndrome-associated mutations in the voltage sensor of I(Ks) channels. (19590188)	Henrion U.... Seebohm G.	2009	KCNQ1
11	Identification of large gene deletions and duplications in KCNQ1 and KCNH2 in patients with long QT syndrome. (18774102)	Eddy C.A.... Shelling A.N.	2008	KCNQ1, KCNH2, KCNE2
12	Drug-induced long QT syndrome in women: review of current evidence and remaining gaps. (18573480)	Hreiche R.... Turgeon J.	2008	KCNH2
13	Mutation analysis of candidate genes SCN1B, KCND3 and ANK2 in patients with clinical diagnosis of long QT syndrome. (18052691)	RaudenskA! M.... Spinar J.	2008	KCND3, KCNE2, ANK2
14	Malignant perinatal variant of long-QT syndrome cause d by a profoundly dysfunctional cardiac sodium channel. (19808432)	Wang D.W.... George A.L.	2008	SCN5A
15	Electrocardiographic interventricular dispersion of repolarization during autonomic adaptation in LQT1 subtype of long QT syndrome. (18365896)	Haapalahti P.... Toivonen L.	2008	KCNQ1
16	Fever-induced QTc prolongation and ventricular arrhythmias in individuals with type 2 congenital long QT syndrome. (18551196)	Amin A.S.... Wilde A.A.	2008	KCNH2
17	Mechanistic basis for the pathogenesis of long QT syndrome associated with a common splicing mutation in KCNQ1 gene. (17292394)	Tsuji K.... Horie M.	2007	KCNQ1
18	A single hERG mutation underlying a spectrum of acquired and congenital long QT syndrome phenotypes. (17531263)	Saenen J.B.... Raes A.L.	2007	KCNH2
19	Prevalence of long-QT syndrome gene variants in sudden infant death syndrome. (17210839)	Arnestad M.... Schwartz P.J.	2007	KCNQ1, KCNH2, KCNE1
20	Effect of physical training on ventricular repolarization in type 1 long QT syndrome: a pilot study in asymptomatic carriers of the G589D KCNQ1 mutation. (16882680)	Perhonen M.A.... Toivonen L.	2006	KCNQ1
21	The heterogeneous spectrum of the long QT syndrome. (16762771)	Patel N.D.... Mathew S.T.	2006	KCNQ1, KCNH2
22	Molecular mechanisms for drug interactions with hERG that cause long QT syndrome. (16863470)	Stansfeld P.J.... Mitcheson J.S.	2006	KCNH2
23	Phenotypic variability and unusual clinical severity of congenital long-QT syndrome in a founder population. (16246960)	Brink P.A.... Schwartz P.J.	2005	KCNQ1
24	Abnormal KCNQ1 trafficking influences disease pathogenesis in hereditary long QT syndromes (LQT1). (15935335)	Wilson A.J.... Tinker A.	2005	KCNQ1
25	An intronic mutation causes long QT syndrome. (15364333)	Zhang L.... Splawski I.	2004	KCNH2
26	Compound mutations: a common cause of severe long-QT syndrome. (15051636)	Westenskow P.... Sanguinetti M.C.	2004	KCNQ1, KCNH2, KCNE1
27	Long QT syndrome and life threatening arrhythmia in a newborn: molecular diagnosis and treatment response. (14676229)	Schulze-Bahr E.... Kehl H.G.	2004	SCN5A
28	Clinical and electrophysiological characterization of a novel mutation (F193L) in the KCNQ1 gene associated with long QT syndrome. (12653681)	Yamaguchi M.... Higashida H.	2003	KCNQ1
29	Altered potassium balance and aldosterone secretion in a mouse model of human congenital long QT syndrome. (11438691)	Arrighi I.... Meneton P.	2001	KCNQ1
30	Linkage and mutation analysis in two Taiwanese families with long QT syndrome. (11802537)	Ko Y.L.... Lin M.W.	2001	KCNQ1, KCNH2, KCNE1
31	Molecular biology and cellular mechanisms of Brugada and long QT syndromes in infants and young children. (11781953)	Antzelevitch C.	2001	KCNQ1, KCNH2, SCN5A
32	Long QT syndrome with a high mortality rate caused by a novel G572R missense mutation in KCNH2. (10735633)	Larsen L.A.... Christiansen M.	2000	KCNH2
33	Long QT syndrome: ionic basis and arrhythmia mechanism in long QT syndrome type 1. (10868746)	Sanguinetti M.C.	2000	KCNQ1
34	Novel KCNQ1 mutations associated with recessive and dominant congenital long QT syndromes: evidence for variable hearing phenotype associated with R518X. (10737999)	Wei J.... George A.L.	2000	KCNQ1
35	Effects of flecainide in patients with new SCN5A mutation: mutation-specific therapy for long-QT syndrome? (10758053)	Benhorin J.... medina A.	2000	SCN5A
36	Characterization of a novel missense mutation in the pore of HERG in a patient with long QT syndrome. (10517660)	Yoshida H.... Sasayama S.	1999	KCNH2
37	Long QT syndrome-associated mutations in the S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunits. (10409658)	Franqueza L.... Sanguinetti M.C.	1999	KCNQ1
38	Congenital long-QT syndrome caused by a novel mutation in a conserved acidic domain of the cardiac Na+ channel. (10377081)	Wei J.... George A.L. Jr.	1999	SCN5A
39	Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation. (10187793)	Chen J.... Sanguinetti M.C.	1999	KCNH2
40	Romano-Ward long QT syndrome: identification of a HER G mutation in a Taiwanese kindred. (10560244)	Lee-Chen G.J.... Teng Y.N.	1999	KCNH2
41	Phenotypic characterization of a novel long-QT syndrome mutation (R1623Q) in the cardiac sodium channel. (9495298)	Kambouris N.G.... Balser J.R.	1998	SCN5A
42	A recessive variant of the Romano-Ward Long-QT syndrome? (9641694)	Priori S.G.... Casari G.	1998	KCNQ1
43	Genomic organization and mutational analysis of HERG, a gene responsible for familial long QT syndrome. (9600240)	Itoh T.... Nakamura Y.	1998	KCNH2
44	New mutations in the KVLQT1 potassium channel that cause long-QT syndrome. (9570196)	Li H.... Towbin J.A.	1998	KCNQ1, KCNH2, SCN5A
45	The electrophysiological mechanism of ventricular arrhythmias in the long QT syndrome. Tridimensional mapping of activation and recovery patterns. (8781481)	el-Sherif N.... Restivo M.	1996	ENPEP
46	The molecular genetics of the congenital long QT syndromes. (8664531)	Russell M.W.... Dick M.	1996	KCNH2
47	A molecular basis for the therapy of the long QT syndrome. (8952843)	Priori S.G.... Schwartz P.J.	1996	KCNH2
48	The long QT syndrome. A review of recent molecular genetic and physiologic discoveries. (8569466)	Keating M.T.	1996	SCN5A
49	Mapping of a gene for long QT syndrome to chromosome 4q25-27. (7485162)	Schott J.J.... Moisan J.P.	1995	ANK2
50	Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity. (7842012)	Jiang C.... Vincent G.M.	1994	KCNH2, SCN5A

Genes related to long qt syndrome according to GeneCards:

(show top 50)    (show all 51)

id	Symbol	Description	Score	GeneCards Section Context
1	SCN5A	sodium channel, voltage-gated, type V, alpha subunit	11.1	Aliases & Descriptions, Publications, Summaries
2	KCNH2	potassium voltage-gated channel, subfamily H (eag-related), member 2	11.1	Summaries, Publications
3	KCNQ1	potassium voltage-gated channel, KQT-like subfamily, member 1	11.1	Summaries, Orthologs, Publications
4	KCNE1	potassium voltage-gated channel, Isk-related family, member 1	11.1	Summaries, Publications
5	ANK2	ankyrin 2, neuronal	11.1	Summaries, Publications
6	SCN4B	sodium channel, voltage-gated, type IV, beta subunit	11.1	Summaries, Publications
7	SNTA1	syntrophin, alpha 1	11.1	Summaries, Publications
8	KCNE2	potassium voltage-gated channel, Isk-related family, member 2	11.0	Publications
9	ALG10	asparagine-linked glycosylation 10, alpha-1,2-glucosyltransferase homolog (S. pombe)	11.0	Disorders
10	KCNJ2	potassium inwardly-rectifying channel, subfamily J, member 2	11.0	Publications
11	CAV3	caveolin 3	11.0	Publications
12	AKAP9	A kinase (PRKA) anchor protein (yotiao) 9	11.0	Publications
13	KCNJ5	potassium inwardly-rectifying channel, subfamily J, member 5	11.0
14	KCND3	potassium voltage-gated channel, Shal-related subfamily, member 3	11.0	Publications
15	MYH7	myosin, heavy chain 7, cardiac muscle, beta	11.0
16	KCND2	potassium voltage-gated channel, Shal-related subfamily, member 2	11.0	Publications
17	RYR2	ryanodine receptor 2 (cardiac)	11.0	Publications
18	SLN	sarcolipin	11.0	Publications
19	KCNQ1OT1	KCNQ1 opposite strand/antisense transcript 1 (non-protein coding)	11.0	Publications
20	KCNE1L	KCNE1-like	11.0	Publications
21	ZACN	zinc activated ligand-gated ion channel	11.0	Publications
22	ALG10B	asparagine-linked glycosylation 10, alpha-1,2-glucosyltransferase homolog B (yeast)	11.0	UniProt Related
23	FKBP1B	FK506 binding protein 1B, 12.6 kDa	11.0	Publications
24	GORASP1	golgi reassembly stacking protein 1, 65kDa	11.0	Publications
25	PTRF	polymerase I and transcript release factor	11.0	Publications
26	KCNE3	potassium voltage-gated channel, Isk-related family, member 3	11.0	Publications
27	EDEM1	ER degradation enhancer, mannosidase alpha-like 1	11.0	Publications
28	SCN1B	sodium channel, voltage-gated, type I, beta subunit	11.0	Publications
29	ADRA2C	adrenoceptor alpha 2C	11.0	Publications
30	GOLGA2	golgin A2	11.0	Publications
31	KCNA5	potassium voltage-gated channel, shaker-related subfamily, member 5	11.0	Publications
32	ADRB1	adrenoceptor beta 1	11.0	Publications
33	BTBD10	BTB (POZ) domain containing 10	11.0	Publications
34	KCNQ2	potassium voltage-gated channel, KQT-like subfamily, member 2	11.0	Publications
35	HCN4	hyperpolarization activated cyclic nucleotide-gated potassium channel 4	11.0	Publications
36	MINK1	misshapen-like kinase 1	11.0	Publications
37	SCN8A	sodium channel, voltage gated, type VIII, alpha subunit	11.0	Publications
38	UPF1	UPF1 regulator of nonsense transcripts homolog (yeast)	11.0	Publications
39	STK11	serine/threonine kinase 11	11.0	Publications
40	ATF6	activating transcription factor 6	11.0	Publications
41	SHH	sonic hedgehog	11.0	Publications
42	ENPEP	glutamyl aminopeptidase (aminopeptidase A)	11.0	Publications
43	NOS1AP	nitric oxide synthase 1 (neuronal) adaptor protein	11.0	Publications
44	HSP90AA1	heat shock protein 90kDa alpha (cytosolic), class A member 1	11.0	Publications
45	KCNQ3	potassium voltage-gated channel, KQT-like subfamily, member 3	11.0	Publications
46	ADRB2	adrenoceptor beta 2, surface	11.0	Publications
47	AKAP13	A kinase (PRKA) anchor protein 13	11.0	Publications
48	NOS1	nitric oxide synthase 1 (neuronal)	11.0	Publications
49	KCNH1	potassium voltage-gated channel, subfamily H (eag-related), member 1	11.0	Publications
50	NSG1	neuron specific gene family member 1	9.9	Publications

Pathways related to long qt syndrome according to GeneDecks:

(show all 14)

id	Pathway	Score	Top Affiliating Genes
1	Potassium transporters- inward current10	10.1	HCN4, KCNJ5, KCNJ2, KCNH2, KCNE1
2	Adrenoceptors38	9.8	ADRB1, ADRB2
3	Sympathetic Nerve Pathway (Neuroeffector Junction)34	9.7	ADRB2, ADRA2C, ADRB1
4	Synaptic transmission- ion currents10	9.7	HCN4, KCNQ3, KCNQ2, KCNJ5, KCND2
5	Aldosterone Signaling in Epithelial Cells36	9.7	HSP90AA1, SCN5A, SCN8A, SCN1B, SCN4B
6	Voltage gated Potassium channels38	9.6	KCNH1, KCND3, KCNQ1, KCNH2, KCND2, KCNA5
7	Neuropathic Pain-Signaling in Dorsal Horn Neurons36	9.4	SCN4B, SCN1B, SCN8A, SCN5A, NOS1
8	PKA Signaling36	9.4	RYR2, AKAP9, AKAP13, SCN5A, SCN8A, SCN1B
9	CFTR folding and maturation (norm and CF)41	9.2	HSP90AA1, CANX, EDEM1
10	Activation of cAMP-Dependent PKA36	8.7	ADRB1, ADRA2C, ADRB2, RYR2, AKAP9, AKAP13
11	cAMP Pathway36	8.7	ADRB1, ADRA2C, ADRB2, RYR2, AKAP9, AKAP13
12	Potassium transporters- outward current10	8.6	KCNQ3, KCNA5, KCND2, KCNQ2, KCNJ5, KCNH1
13	Antiarrhythmic Pathway, Pharmacodynamics34	8.2	KCNJ5, KCNE2, KCND3, KCNQ1, KCNJ2, KCNH2
14	Dopamine-DARPP32 Feedback onto cAMP Pathway36	7.9	AKAP9, KCNQ3, KCNQ2, KCNJ5, KCNH1, KCNE2

Compounds related to long qt syndrome according to GeneDecks:

(show top 50)    (show all 58)

id	Compound	Score	Top Affiliating Genes
1	chromanol32	10.6	KCNQ1, KCNE1
2	flecainide32 34 9 9	13.6	KCND2, KCNH2, SCN5A
3	disopyramide32 9 9	12.5	KCND2, KCND3, KCNJ5, SCN5A
4	clofilium32	10.5	KCNQ1, KCNH2
5	flecainide acetate42	10.4	SCN5A, SCN8A, SCN1B, SCN4B
6	ralfinamide mesylate42	10.4	SCN5A, SCN8A, SCN1B, SCN4B
7	qx 314 chloride42	10.4	SCN5A, SCN8A, SCN1B, SCN4B
8	tetrodotoxin citrate42	10.4	SCN5A, SCN8A, SCN1B, SCN4B
9	vinpocetine32 42	11.3	SCN5A, SCN8A, SCN1B, SCN4B
10	xe 991 dihydrochloride42	10.2	KCNQ3, KCNQ2, KCNQ1
11	linopirdine dihydrochloride42	10.2	KCNQ3, KCNQ2, KCNQ1
12	Pirbuterol9 9	11.2	ADRB2, ADRB1
13	Levobunolol9 9	11.2	ADRB2, ADRB1
14	sotalol32 9 9	12.2	ADRB1, ADRB2, KCNH2
15	Metipranolol9 9	11.2	ADRB1, ADRB2
16	isoleucine32	10.2	KCNH2, KCNQ1, SCN5A, ADRB2
17	metoprolol32 34 9 18 9	14.2	KCNH2, ADRB2, ADRB1
18	aprikalim32	10.2	NOS1, KCNJ5
19	atenolol32 9 18 9	13.2	ADRB1, ADRB2, KCNH2
20	Bevantolol9 9	11.1	ADRB1, ADRB2
21	Arbutamine9 9	11.1	ADRB1, ADRB2
22	chromanol 293b42 32	11.1	KCNH1, KCNQ1, KCNE1
23	dopexamine32	10.1	ADRB1, ADRB2
24	Droxidopa9 9	11.1	ADRB2, ADRA2C, ADRB1
25	Ephedra9 9	11.1	ADRB1, ADRA2C, ADRB2
26	Oxprenolol9 9	11.0	ADRB1, ADRB2
27	carvedilol32 34 9 9	13.0	ADRB1, ADRB2, RYR2, FKBP1B, KCNH2
28	nadolol32 9 9	11.9	ADRB1, ADRB2
29	bupranolol32 9 9	11.9	ADRB1, ADRB2
30	phenylephrine32 9 18 9	12.8	MYH7, ADRB1, NOS1, CAV3
31	icyp32	9.8	ADRB1, ADRB2
32	valine32	9.7	MYH7, ADRB1, NOS1, SCN5A, KCNQ2, KCNH2
33	tetraethylammonium32	9.7	NOS1, KCNQ3, KCNJ5, KCNQ1, KCNJ2, KCNH2
34	penbutolol32 9 9	11.6	ADRB1, ADRB2
35	ryanodine32	9.5	MYH7, RYR2, AKAP13, CANX, ANK2, FKBP1B
36	inositol 1,4,5 trisphosphate32	9.5	RYR2, AKAP9, AKAP13, CANX, ANK2
37	tacrolimus32 34 9 9	12.1	RYR2, NOS1, AKAP13, HSP90AA1, FKBP1B
38	kn-9342	9.0	KCNQ3, KCNQ2, KCNH1, KCNE2, KCND3, KCNE3
39	am 92016 hydrochloride42	9.0	KCNQ3, KCNQ2, KCNH1, KCNE2, KCND3, KCNQ1
40	4-aminopyridine32 42	10.0	KCNQ3, KCNQ2, KCNH1, KCNE2, KCND3, KCNH2
41	adenylate32	8.8	ADRB1, ADRB2, STK11, NOS1, HSP90AA1, KCNJ5
42	isoproterenol32 9 9	10.7	MYH7, ADRB1, ADRB2, RYR2, NOS1, AKAP13
43	nitric oxide32 9 18 9	11.6	ADRB1, ADRB2, RYR2, NOS1AP, NOS1, SNTA1
44	cyclic amp32 18	9.3	ADRB1, ADRB2, RYR2, STK11, HCN4, AKAP9
45	sodium32 18	9.2	SNTA1, CAV3, ENPEP, ZACN, SCN5A, SCN8A
46	arginine32	8.2	AKAP13, NOS1, ADRB2, ADRB1, MYH7, HSP90AA1
47	lipid32	8.1	STK11, SLN, RYR2, ADRB2, ADRB1, MYH7
48	glutamate32	7.9	ADRB1, ADRB2, RYR2, NOS1, AKAP13, HSP90AA1
49	calcium32 9 18 9	9.9	CANX, CAV3, AKAP13, SNTA1, NOS1, STK11
50	potassium32 9 18 9	9.4	BTBD10, KCNJ5, KCNH1, KCNE2, KCND3, KCNQ1

Cellular components related to long qt syndrome according to GeneDecks:

(show all 11)

id	Name	GO ID	Score	Top Affiliating Genes
1	intercalated disc	GO:014704	10.2	KCNA5, SCN4B, SCN5A, ANK2
2	T-tubule	GO:030315	10.2	KCNJ5, SCN5A, ANK2, CAV3
3	voltage-gated sodium channel complex	GO:001518	10.1	SCN5A, SCN8A, SCN1B, SCN4B
4	Z disc	GO:030018	9.9	MYH7, RYR2, CAV3, ANK2, SCN8A
5	sarcoplasmic reticulum	GO:016529	9.7	NOS1, SLN, RYR2
6	caveola	GO:005901	9.7	ADRB2, PTRF, CAV3, SCN5A, KCNA5
7	axon initial segment	GO:043194	9.6	KCNQ2, KCNQ3, SCN8A
8	node of Ranvier	GO:033268	9.4	SCN8A, KCNQ3, KCNQ2
9	sarcolemma	GO:042383	9.3	ADRB2, NOS1, SNTA1, CAV3, ANK2, SCN5A
10	voltage-gated potassium channel complex	GO:008076	8.6	KCNA5, KCNQ3, KCNQ2, KCNJ5, KCNH1, KCNE2
11	plasma membrane	GO:005886	6.2	MINK1, ANK2, ALG10B, HSP90AA1, CAV3, SHH

Biological processes related to long qt syndrome according to GeneDecks:

(show all 31)

id	Name	GO ID	Score	Top Affiliating Genes
1	T-tubule organization	GO:033292	10.6	ANK2, CAV3
2	regulation of ventricular cardiac muscle cell action potential	GO:086005	10.6	RYR2, ANK2, SCN5A
3	AV node cell to bundle of His cell communication	GO:086067	10.6	SCN4B, SCN5A
4	SA node cardiomyocyte to atrial cardiomyocyte communication	GO:086070	10.5	SCN5A, ANK2
5	sarcoplasmic reticulum calcium ion transport	GO:070296	10.5	RYR2, ANK2
6	membrane depolarization involved in regulation of cardiac muscle cell action potential	GO:086012	10.5	SCN5A, SCN1B, SCN4B
7	regulation of cardiac muscle cell action potential involved in contraction	GO:086002	10.5	SCN5A, SCN1B, SCN4B
8	potassium ion export	GO:071435	10.5	KCNQ1, KCNE1, KCNA5
9	regulation of heart rate	GO:002027	10.5	SCN5A, ANK2, CAV3, MYH7
10	regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ion	GO:010881	10.5	FKBP1B, ANK2, RYR2
11	regulation of sodium ion transmembrane transporter activity	GO:2000649	10.5	CAV3, SCN1B, SCN4B
12	sodium ion transmembrane transport	GO:035725	10.4	SCN5A, SCN1B, SCN4B
13	membrane depolarization	GO:051899	10.4	KCNE1, SCN1B, SCN5A
14	regulation of heart rate by cardiac conduction	GO:086091	10.4	ANK2, SCN5A, SCN1B, SCN4B, KCNH2
15	regulation of ventricular cardiomyocyte membrane repolarization	GO:060307	10.4	CAV3, ANK2, SCN5A, SCN1B, SCN4B
16	cardiac muscle contraction	GO:060048	10.4	RYR2, SCN5A, SCN1B, SCN4B
17	blood circulation	GO:008015	10.3	HCN4, KCNE2, KCNQ1, KCNH2, KCNE1
18	regulation of ventricular cardiomyocyte membrane depolarization	GO:060373	10.3	SCN5A, CAV3
19	release of sequestered calcium ion into cytosol by sarcoplasmic reticulum	GO:014808	10.2	FKBP1B, RYR2
20	regulation of heart contraction	GO:008016	10.1	HCN4, CAV3, KCNE2, KCNQ1, KCNH2, KCNE1L
21	regulation of cardiac muscle contraction	GO:055117	10.1	NOS1, CAV3, ANK2
22	negative regulation of smooth muscle contraction	GO:045986	10.1	ADRB1, ADRB2
23	regulation of membrane potential	GO:042391	10.0	CAV3, KCNH2, KCNA5
24	heat generation	GO:031649	10.0	ADRB1, ADRB2
25	muscle contraction	GO:006936	10.0	MYH7, HCN4, SNTA1, FKBP1B, KCNE2, KCNQ1
26	vasodilation by norepinephrine-epinephrine involved in regulation of systemic arterial blood pressure	GO:002025	10.0	ADRB1, ADRB2
27	protein N-linked glycosylation via asparagine	GO:018279	9.6	EDEM1, CANX, ALG10B, ALG10
28	diet induced thermogenesis	GO:002024	9.6	ADRB2, ADRB1
29	axon guidance	GO:007411	9.1	SHH, HSP90AA1, ANK2, SCN8A, SCN1B, KCNQ3
30	potassium ion transport	GO:006813	9.0	KCNQ3, KCNQ2, KCNJ5, KCNH1, KCNE2, KCND3
31	synaptic transmission	GO:007268	8.3	KCNA5, HCN4, AKAP9, MINK1, SCN1B, KCNQ3

Molecular functions related to long qt syndrome according to GeneDecks:

(show all 14)

id	Name	GO ID	Score	Top Affiliating Genes
1	voltage-gated sodium channel activity involved in regulation of cardiac muscle cell action potential	GO:086006	10.4	SCN5A, SCN1B, SCN4B
2	ion channel binding	GO:044325	10.3	SCN4B, SCN5A, ANK2, CAV3
3	inward rectifier potassium channel activity	GO:005242	10.2	KCNH2, KCNJ2, KCNJ5
4	voltage-gated sodium channel activity	GO:005248	10.2	SCN4B, SCN8A, SCN5A
5	sodium channel regulator activity	GO:017080	10.2	CAV3, SCN1B, SCN4B
6	alpha-2A adrenergic receptor binding	GO:031694	10.1	ADRA2C, ADRB1
7	potassium channel regulator activity	GO:015459	10.0	KCNE1, KCNE3, KCNE2, ADRB2
8	ryanodine-sensitive calcium-release channel activity	GO:005219	9.9	FKBP1B, RYR2
9	epinephrine binding	GO:051379	9.9	ADRB1, ADRA2C, ADRB2
10	norepinephrine binding	GO:051380	9.8	ADRB1, ADRB2
11	delayed rectifier potassium channel activity	GO:005251	9.7	KCNH1, KCNQ1, KCNH2, KCNE1, KCNA5
12	voltage-gated potassium channel activity	GO:005249	9.4	KCND2, HCN4, KCNQ3, KCNQ2, KCNE2, KCNQ1
13	calmodulin binding	GO:005516	9.1	MYH7, RYR2, NOS1, SNTA1, SCN5A, KCNH1
14	protein binding	GO:005515	4.7	AKAP9, PTRF, SNTA1, NOS1, SHH, EDEM1