KCNJ2

Summary

KCNJ2 (potassium inwardly rectifying channel subfamily J member 2, HGNC:6263) is a protein-coding gene on chromosome 17q24.3, encoding Inward rectifier potassium channel 2 (P63252). Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it.

Potassium channels are present in most mammalian cells, where they participate in a wide range of physiologic responses. The protein encoded by this gene is an integral membrane protein and inward-rectifier type potassium channel. The encoded protein, which has a greater tendency to allow potassium to flow into a cell rather than out of a cell, probably participates in establishing action potential waveform and excitability of neuronal and muscle tissues. Mutations in this gene have been associated with Andersen syndrome, which is characterized by periodic paralysis, cardiac arrhythmias, and dysmorphic features.

Source: NCBI Gene 3759 — RefSeq curated summary.

At a glance

• Gene–disease (curated): Andersen-Tawil syndrome (Definitive, GenCC) — +7 more curated relationships
• GWAS associations: 120
• Clinical variants (ClinVar): 684 total — 42 pathogenic, 19 likely-pathogenic
• Phenotypes (HPO): 98
• Druggable target: yes
• MANE Select transcript: NM_000891

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:6263
Approved symbol	KCNJ2
Name	potassium inwardly rectifying channel subfamily J member 2
Location	17q24.3
Locus type	gene with protein product
Status	Approved
Aliases	Kir2.1, IRK1, LQT7
Ensembl gene	ENSG00000123700
Ensembl biotype	protein_coding
OMIM	600681
Entrez	3759

Gene structure

Transcript identifiers

Ensembl transcripts: 4 — 4 protein_coding

ENST00000243457, ENST00000535240, ENST00000854891, ENST00000854892

RefSeq mRNA: 1 — MANE Select: NM_000891 NM_000891

CCDS: CCDS11688

Canonical transcript exons

ENST00000243457 — 2 exons

Exon	Start	End
ENSE00000841562	70174824	70180044
ENSE00001012579	70169532	70169701

Expression profiles

Bgee: expression breadth ubiquitous, 256 present calls, max score 95.99.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 8.3691 / max 352.1758, expressed in 1119 samples.

FANTOM5 promoters (4 alternative TSS)

Promoter ID	TPM avg	Samples expressed
162505	7.4052	1077
162506	0.6541	240
162503	0.2477	98
162504	0.0622	20

Top tissues by expression

284 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
inferior vagus X ganglion	UBERON:0005363	95.99	gold quality
skeletal muscle tissue of rectus abdominis	UBERON:0004511	95.71	gold quality
dorsal motor nucleus of vagus nerve	UBERON:0002870	94.29	gold quality
skeletal muscle tissue of biceps brachii	UBERON:0004502	94.00	gold quality
biceps brachii	UBERON:0001507	93.52	gold quality
pons	UBERON:0000988	93.50	gold quality
medulla oblongata	UBERON:0001896	93.07	gold quality
left ventricle myocardium	UBERON:0006566	92.44	gold quality
subthalamic nucleus	UBERON:0001906	92.09	gold quality
corpus callosum	UBERON:0002336	91.87	gold quality
superior vestibular nucleus	UBERON:0007227	91.78	gold quality
inferior olivary complex	UBERON:0002127	91.50	gold quality
body of tongue	UBERON:0011876	91.06	gold quality
vastus lateralis	UBERON:0001379	90.79	gold quality
deltoid	UBERON:0001476	90.55	gold quality
ventral tegmental area	UBERON:0002691	90.53	gold quality
quadriceps femoris	UBERON:0001377	90.50	gold quality
cranial nerve II	UBERON:0000941	90.40	gold quality
medial globus pallidus	UBERON:0002477	89.75	gold quality
globus pallidus	UBERON:0001875	89.61	gold quality
tibialis anterior	UBERON:0001385	89.48	gold quality
bronchial epithelial cell	CL:0002328	89.02	gold quality
epithelium of bronchus	UBERON:0002031	88.86	gold quality
skeletal muscle tissue	UBERON:0001134	88.32	gold quality
bronchus	UBERON:0002185	88.29	gold quality
epithelium of nasopharynx	UBERON:0001951	87.56	gold quality
diaphragm	UBERON:0001103	87.52	silver quality
endothelial cell	CL:0000115	87.20	gold quality
dorsal plus ventral thalamus	UBERON:0001897	87.17	gold quality
blood	UBERON:0000178	86.41	gold quality

Single-cell (SCXA)

Detected in 7 experiment(s), a significant marker in 7.

Experiment	Marker?	Max mean expression
E-GEOD-135922	yes	400.23
E-HCAD-10	yes	35.80
E-MTAB-5061	yes	9.02
E-ANND-3	yes	8.41
E-GEOD-137537	yes	5.02
E-GEOD-81608	yes	4.94
E-GEOD-81547	yes	4.68

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): E2F1, SP1, SP3

miRNA regulators (miRDB)

241 targeting KCNJ2, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-9-5P	100.00	72.28	2361
HSA-MIR-3163	100.00	77.23	8605
HSA-MIR-1277-5P	100.00	73.95	5056
HSA-MIR-3613-3P	100.00	76.36	7965
HSA-MIR-196A-5P	100.00	68.16	684
HSA-MIR-196B-5P	100.00	68.16	681
HSA-MIR-5692A	100.00	74.40	6850
HSA-MIR-4673	100.00	66.64	1490
HSA-MIR-5011-5P	100.00	83.46	5820
HSA-MIR-5692B	100.00	71.32	2622
HSA-MIR-5692C	100.00	71.32	2622
HSA-MIR-548AW	99.99	72.57	3559
HSA-MIR-4282	99.99	75.36	6408
HSA-MIR-19A-3P	99.98	75.33	2762
HSA-MIR-19B-3P	99.98	75.44	2754
HSA-MIR-4775	99.98	75.00	6394
HSA-MIR-5696	99.98	72.36	4487
HSA-MIR-4645-5P	99.98	65.81	1284
HSA-MIR-607	99.97	73.62	5593
HSA-MIR-302C-5P	99.97	72.56	3642
HSA-MIR-3148	99.97	75.06	6478
HSA-MIR-1468-3P	99.96	72.74	3797
HSA-MIR-548AJ-3P	99.96	73.38	5345
HSA-MIR-548X-3P	99.96	73.38	5345
HSA-MIR-9-3P	99.96	70.88	2068
HSA-MIR-551B-5P	99.96	71.28	3493
HSA-MIR-570-3P	99.96	72.41	4910
HSA-MIR-495-3P	99.96	72.81	4197
HSA-MIR-5688	99.96	73.23	4504
HSA-MIR-559	99.95	72.28	3609

Literature-anchored findings (GeneRIF, showing 40)

• molecular cloning from and conductance in nasal mucosal epithelium (PMID:11688996)
• Modulation of the inward rectifier potassium channel IRK1 by the Ras signaling pathway (PMID:11809752)
• Heteromerization of Kir2.1 channel contributes to the phenotype of Andersen syndrome (PMID:12032359)
• Thr192Ala missense mutation found in familial periodic paralysis with ventricular dysrhythmia and marked QT prolongation (PMID:12045162)
• KCNJ2 mutation results in Andersen syndrome with sex-specific cardiac and skeletal muscle phenotypes (PMID:12148092)
• Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome) (PMID:12163457)
• effect of suppressing excitability in single neurons within a network of active hippocampal neurons by overexpressing an inward-rectifier potassium channel (PMID:12459783)
• expression of Kir2.1 protein in proliferative smooth muscle cells, consistent with the higher current density (PMID:12598232)
• filamin-A was found to have no effect on Kir2.1 channel behavior but, rather, increased the number of functional channels resident within the membrane (PMID:12923176)
• the small GTPase, Rho, transduces the m1 muscarinic receptor-induced inhibition of Kir2.1 via an unidentified mechanism. (PMID:14500755)
• data show that the Andersen-Tawil syndrome phenotype may occur through a dominant-negative effect as well as through haplo-insufficiency and reveal amino acids critical in trafficking and conductance of the inward rectifier K+ channels. (PMID:14522976)
• Kir2.1 channel activation is a required key early event that initiates myogenesis by turning on myogenin and MEF2 transcription factors via a hyperpolarization-activated Ca(2+)-dependent pathway (PMID:15084602)
• Coexpression of Kir2.1 and PSD-93delta had no discernible effect upon channel kinetics but resulted in cell surface Kir2.1 clustering and suppression of channel internalization. (PMID:15304517)
• Data describe the construction of a new cell line stably expressing alpha(1G) and Kir2.1 subunits in HEK293 cells. (PMID:15465033)
• The present results suggest that the outward I(K1) flows through two populations of Kir2.1 channels with different sensitivities to cytoplasmic blockers. (PMID:15618275)
• Consequently, the steady-state voltage dependence of IRK1 block by spermine or bis-QA(C10) should increase with membrane depolarization, a prediction indeed observed. (PMID:15795311)
• Andersen’s syndrome-associated mutations and hypokalaemic periodic paralysis-associated calcium channel mutations may lead to muscle membrane hypoexcitability via a common mechanism (PMID:15831539)
• Kir2.1 gain-of-function mutation may have a role in development of familial atrial fibrillation (PMID:15922306)
• Kir2.2 and Kir2.1 are primary determinants of endogenous K(+) conductance in HAECs under resting conditions and that Kir2.2 provides the dominant conductance in these cells. (PMID:15958527)
• Results describe the regulation of inwardly rectifying potassium current and its main molecular correlates, Kir2.1, Kir2.2 and Kir2.3 channels, by endothelin-1 in human atrial cardiomyocytes. (PMID:16258766)
• In conclusion, the data are consistent with the universal mechanism of rectification in Kir2 channels, but also point to significant, and physiologically important, quantitative differences between Kir2 isoforms. (PMID:16373386)
• Results suggest that chronic exposure to certain drugs and their effects on Kir2.1 and ERG potassium channels may be an important aspect of acquired QT prolongation. (PMID:16407206)
• Mutations in KCNJ2 is associated with Andersen-Tawil syndrome (PMID:16419128)
• The cytoplasmic pore of Kir electrostatically gathers cations such as Mg(2+), spermine, and K(+) so that the transmembrane pore is sufficiently filled with K(+) ions, which enables strong voltage-dependent blockade with adequate outward K(+) conductance. (PMID:16533896)
• The results demonstrate functional consequences of two novel trafficking-competent KCNJ2 mutations associated with Andersen syndrome. (PMID:16541386)
• Kir2.1 had a strong dominant negative effect in the Xenopus oocyte expression system. The T75R-Transgenic mice had bidirectional ventricular tachycardia after induction and longer QT intervals. (PMID:16571646)
• These results establish the direct regulation of Kir channels by the cytoplasmic accumulation of LC-CoA, which might be of physiological and pathophysiological relevance in a variety of tissues. (PMID:16777940)
• Kir2.1-induced hyperpolarization triggers human myoblast differentiation via the activation of the calcineurin pathway, which, in turn, induces expression/activity of myogenin and MEF2. (PMID:16831831)
• Individual Andersen’s Syndrome mutations R218Q, G300V, E303K, and delta314-315 affecting the ability of the cytoplasmic domains in Kir2.1 channels to form proper tetrameric assemblies were characterised. (PMID:16834334)
• Co-expression of Kir2.1 changes the pattern of subcellular distribution of GRIF-1. (PMID:16895905)
• We demonstrated that 9.5% of cases diagnosed as SIDS carry functionally significant genetic variants in LQTS genes (KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, KCNJ2, CAV3). (PMID:17210839)
• Two missense mutations of KCNJ2 (R218Q and M307I) are identified in two Korean families diagnosed with Andersen-Tawil syndrome. (PMID:17211524)
• Novel Kir2.1 mutations at residues C54 and T305 linked to Andersen Syndrome. (PMID:17324964)
• KCNJ2 loss of function mutations were found in approximately 1% of patients referred for genetic arrhythmia testing that lacked criteria for Andersen-Tawil Syndrome. (PMID:17341397)
• These findings suggest that non-syndromic PRS may be caused by both SOX9 and KCNJ2 dysregulation. (PMID:17551083)
• The T75M mutation caused alteration of gating kinetics of the mutated KCNJ2 channels, i.e., increased sensitivity to intracellular Mg2+ and resultant enhancement of inward rectification. (PMID:17582433)
• results suggest that 1 negative charge of D152 or 2 negative charges of E153 are required for Kir2.1 channels to function. contribution by D152 & E153 to the electronegative extracellular pore entrance is critical for the channel to function properly. (PMID:17619200)
• Modulation of the outward Kir2.1 current alters tone and calcium signaling in the afferent arterioles but not in the efferent arterioles of kidneys. (PMID:18178799)
• We conclude that the lysosomal degradation pathway contributes to Kir2.1 mediated inward rectifier current regulation. (PMID:18182162)
• Kir2.1 channels are already present at the membrane of proliferating, undifferentiated human myoblasts but in a silent state, and Kir2.1 tyrosine 242 dephosphorylation triggers differentiation. (PMID:18216177)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	kcnj2a	ENSDARG00000019418
danio_rerio	kcnj2b	ENSDARG00000104389
mus_musculus	Kcnj2	ENSMUSG00000041695
rattus_norvegicus	Kcnj2	ENSRNOG00000064933

Paralogs (15): KCNJ13 (ENSG00000115474), KCNJ5 (ENSG00000120457), KCNJ8 (ENSG00000121361), KCNJ1 (ENSG00000151704), KCNJ16 (ENSG00000153822), KCNJ6 (ENSG00000157542), KCNJ15 (ENSG00000157551), KCNJ9 (ENSG00000162728), KCNJ3 (ENSG00000162989), KCNJ4 (ENSG00000168135), KCNJ10 (ENSG00000177807), KCNJ14 (ENSG00000182324), KCNJ12 (ENSG00000184185), KCNJ11 (ENSG00000187486), KCNJ18 (ENSG00000260458)

Protein

Protein identifiers

Inward rectifier potassium channel 2 — P63252 (reviewed: P63252)

Alternative names: Cardiac inward rectifier potassium channel, Inward rectifier K(+) channel Kir2.1, Potassium channel, inwardly rectifying subfamily J member 2

All UniProt accessions (1): P63252

UniProt curated annotations — full annotation on UniProt →

Function. Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. Their voltage dependence is regulated by the concentration of extracellular potassium; as external potassium is raised, the voltage range of the channel opening shifts to more positive voltages. The inward rectification is mainly due to the blockage of outward current by internal magnesium. Can be blocked by extracellular barium or cesium. Probably participates in establishing action potential waveform and excitability of neuronal and muscle tissues.

Subunit / interactions. Homotetramer. Homomultimeric and heteromultimeric association with KCNJ4/Kir2.3. Can form heteromeric channels with Kir2.6/KCNJ18. Associates, via its PDZ-recognition domain, with a complex containing LIN7A, LIN7B, LIN7C, DLG1, CASK and APBA1.

Subcellular location. Cell membrane. Sarcolemma. T-tubule.

Tissue specificity. Heart, brain, placenta, lung, skeletal muscle, and kidney. Diffusely distributed throughout the brain.

Post-translational modifications. S-nitrosylation increases the open probability and inward rectifying currents.

Disease relevance. Long QT syndrome 7 (LQT7) [MIM:170390] A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Long QT syndrome type 7 manifests itself as a clinical triad consisting of potassium-sensitive periodic paralysis, ventricular ectopy and dysmorphic features. The disease is caused by variants affecting the gene represented in this entry. Short QT syndrome 3 (SQT3) [MIM:609622] A form of short QT syndrome, a heart disorder characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. It can cause syncope and sudden death. SQT3 has a unique ECG phenotype characterized by asymmetrical T waves. The disease is caused by variants affecting the gene represented in this entry. Atrial fibrillation, familial, 9 (ATFB9) [MIM:613980] A familial form of atrial fibrillation, a common sustained cardiac rhythm disturbance. Atrial fibrillation is characterized by disorganized atrial electrical activity and ineffective atrial contraction promoting blood stasis in the atria and reduces ventricular filling. It can result in palpitations, syncope, thromboembolic stroke, and congestive heart failure. The disease is caused by variants affecting the gene represented in this entry.

Activity regulation. Channel opening is promoted by binding phosphatidylinositol-4,5-bisphosphate (PIP2).

Similarity. Belongs to the inward rectifier-type potassium channel (TC 1.A.2.1) family. KCNJ2 subfamily.

RefSeq proteins (1): NP_000882* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR003271	K_chnl_inward-rec_Kir2.1	Family
IPR013518	K_chnl_inward-rec_Kir_cyto	Homologous_superfamily
IPR013673	K_chnl_inward-rec_Kir_N	Domain
IPR014756	Ig_E-set	Homologous_superfamily
IPR016449	K_chnl_inward-rec_Kir	Family
IPR040445	Kir_TM	Domain
IPR041647	IRK_C	Domain

Pfam: PF01007, PF08466, PF17655

Catalyzed reactions (Rhea), 1 shown:

• K(+)(in) = K(+)(out) (RHEA:29463)

UniProt features (35 total): sequence variant 14, topological domain 4, region of interest 2, short sequence motif 2, transmembrane region 2, sequence conflict 2, intramembrane region 2, chain 1, binding site 1, site 1, modified residue 1, disulfide bond 1, mutagenesis site 1, strand 1

Structure

Experimental structures (PDB)

3 structures.

PDB	Method	Resolution (Å)
6SPZ	X-RAY DIFFRACTION	2.08
7ZDZ	ELECTRON MICROSCOPY	4.3
8QQL	ELECTRON MICROSCOPY	6

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-P63252-F1	82.15	0.66

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Catalytic / active sites (1): 172 (role in the control of polyamine-mediated channel gating and in the blocking by intracellular magnesium)

Ligand- & substrate-binding residues (1): 255

Post-translational modifications (1): 76

Disulfide bonds (1): 122–154

Mutagenesis-validated functional residues (1):

Position	Phenotype
312	does not significantly alter affinity for pip2, but mutant channels do not open despite binding pip2.

Function

Pathways and Gene Ontology

Reactome pathways

18 pathways

ID	Pathway
R-HSA-1296041	Activation of G protein gated Potassium channels
R-HSA-1296053	Classical Kir channels
R-HSA-5576886	Phase 4 - resting membrane potential
R-HSA-9729555	Sensory perception of sour taste
R-HSA-997272	Inhibition of voltage gated Ca2+ channels via Gbeta/gamma subunits
R-HSA-112314	Neurotransmitter receptors and postsynaptic signal transmission
R-HSA-112315	Transmission across Chemical Synapses
R-HSA-112316	Neuronal System
R-HSA-1296059	G protein gated Potassium channels
R-HSA-1296065	Inwardly rectifying K+ channels
R-HSA-1296071	Potassium Channels
R-HSA-397014	Muscle contraction
R-HSA-5576891	Cardiac conduction
R-HSA-9709957	Sensory Perception
R-HSA-9717189	Sensory perception of taste
R-HSA-977443	GABA receptor activation
R-HSA-977444	GABA B receptor activation
R-HSA-991365	Activation of GABAB receptors

MSigDB gene sets: 601 (showing top): GSE18804_SPLEEN_MACROPHAGE_VS_BRAIN_TUMORAL_MACROPHAGE_DN, GOBP_POTASSIUM_ION_TRANSPORT, GOBP_MEMBRANE_DEPOLARIZATION, GOBP_CIRCULATORY_SYSTEM_PROCESS, GOBP_PROTEIN_HOMOTETRAMERIZATION, TGCTGCT_MIR15A_MIR16_MIR15B_MIR195_MIR424_MIR497, GOBP_CELLULAR_RESPONSE_TO_EXTERNAL_STIMULUS, REACTOME_POTASSIUM_CHANNELS, REACTOME_INWARDLY_RECTIFYING_K_CHANNELS, GCANCTGNY_MYOD_Q6, GOBP_MEMBRANE_DEPOLARIZATION_DURING_ACTION_POTENTIAL, MODULE_64, IVANOVA_HEMATOPOIESIS_LATE_PROGENITOR, GOBP_POTASSIUM_ION_HOMEOSTASIS, GOBP_RELAXATION_OF_CARDIAC_MUSCLE

GO Biological Process (23): potassium ion transport (GO:0006813), regulation of skeletal muscle contraction via regulation of action potential (GO:0014861), magnesium ion transport (GO:0015693), intracellular potassium ion homeostasis (GO:0030007), regulation of monoatomic ion transmembrane transport (GO:0034765), protein homotetramerization (GO:0051289), relaxation of cardiac muscle (GO:0055119), regulation of resting membrane potential (GO:0060075), regulation of membrane repolarization (GO:0060306), cellular response to mechanical stimulus (GO:0071260), potassium ion transmembrane transport (GO:0071805), cardiac muscle cell action potential involved in contraction (GO:0086002), regulation of cardiac muscle cell contraction (GO:0086004), membrane repolarization during action potential (GO:0086011), membrane depolarization during cardiac muscle cell action potential (GO:0086012), membrane repolarization during cardiac muscle cell action potential (GO:0086013), regulation of heart rate by cardiac conduction (GO:0086091), relaxation of skeletal muscle (GO:0090076), positive regulation of potassium ion transmembrane transport (GO:1901381), potassium ion import across plasma membrane (GO:1990573), monoatomic ion transport (GO:0006811), monoatomic ion transmembrane transport (GO:0034220), cardiac muscle cell action potential (GO:0086001)

GO Molecular Function (5): inward rectifier potassium channel activity (GO:0005242), phosphatidylinositol-4,5-bisphosphate binding (GO:0005546), identical protein binding (GO:0042802), voltage-gated potassium channel activity involved in cardiac muscle cell action potential repolarization (GO:0086008), protein binding (GO:0005515)

GO Cellular Component (11): plasma membrane (GO:0005886), voltage-gated potassium channel complex (GO:0008076), intercalated disc (GO:0014704), membrane (GO:0016020), T-tubule (GO:0030315), neuronal cell body (GO:0043025), dendritic spine (GO:0043197), postsynaptic membrane (GO:0045211), glutamatergic synapse (GO:0098978), dendrite (GO:0030425), monoatomic ion channel complex (GO:0034702)

Reactome top-level categories

Rollup of top-13 pathways:

Category	Pathways
Inwardly rectifying K+ channels	2
Neuronal System	2
G protein gated Potassium channels	1
Cardiac conduction	1
Sensory perception of taste	1
Activation of GABAB receptors	1
Transmission across Chemical Synapses	1
Potassium Channels	1
Muscle contraction	1
Sensory Perception	1
Neurotransmitter receptors and postsynaptic signal transmission	1
GABA receptor activation	1
GABA B receptor activation	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cardiac muscle cell action potential	3
metal ion transport	2
relaxation of muscle	2
regulation of membrane potential	2
membrane repolarization	2
cardiac muscle cell contraction	2
potassium ion transmembrane transport	2
voltage-gated potassium channel activity	2
cellular anatomical structure	2
postsynapse	2
regulation of skeletal muscle contraction	1
regulation of action potential	1
regulation of skeletal muscle contraction by action potential	1
intracellular monoatomic cation homeostasis	1
potassium ion homeostasis	1
monoatomic ion transmembrane transport	1
regulation of transmembrane transport	1
regulation of monoatomic ion transport	1
protein homooligomerization	1
protein tetramerization	1
regulation of biological process	1
response to mechanical stimulus	1
cellular response to abiotic stimulus	1
cellular response to external stimulus	1
potassium ion transport	1
monoatomic cation transmembrane transport	1
regulation of cardiac muscle contraction	1
regulation of actin filament-based movement	1
action potential	1
membrane depolarization during action potential	1
membrane repolarization during action potential	1
cardiac muscle cell membrane repolarization	1
regulation of heart rate	1
cardiac conduction	1
positive regulation of potassium ion transport	1
regulation of potassium ion transmembrane transport	1
positive regulation of cation transmembrane transport	1
inorganic cation import across plasma membrane	1
ligand-gated monoatomic cation channel activity	1
phosphatidylinositol phosphate binding	1

Protein interactions and networks

STRING

0 interactions, top by confidence (×1000):

IntAct

29 interactions, top by confidence:

A	B	Type	Score
KCNJ2	ARL13B	psi-mi:“MI:0915”(physical association)	0.660
KCNJ18	KCNJ2	psi-mi:“MI:0915”(physical association)	0.660
KCNJ2	KCNJ18	psi-mi:“MI:2364”(proximity)	0.660
SCRIB	KCNJ2	psi-mi:“MI:0407”(direct interaction)	0.570
KCNJ2	KCNJ15	psi-mi:“MI:0915”(physical association)	0.560
SDC3	KCNJ2	psi-mi:“MI:0915”(physical association)	0.560
KCNJ2	SEC22A	psi-mi:“MI:0915”(physical association)	0.560
KCNJ2	KCNJ2	psi-mi:“MI:0915”(physical association)	0.560
SDCBP	KCNJ2	psi-mi:“MI:0915”(physical association)	0.560
AKAP5	KCNJ2	psi-mi:“MI:0915”(physical association)	0.540
DLG1	KCNJ2	psi-mi:“MI:0407”(direct interaction)	0.440
KCNJ2	FLNA	psi-mi:“MI:0915”(physical association)	0.400
KCNJ2	ELAPOR2	psi-mi:“MI:2364”(proximity)	0.270
KCNJ2	PIK3R2	psi-mi:“MI:2364”(proximity)	0.270
SEC22A	KCNJ2	psi-mi:“MI:0915”(physical association)	0.000
KCNJ2	KCNJ2	psi-mi:“MI:0915”(physical association)	0.000
SDCBP	KCNJ2	psi-mi:“MI:0915”(physical association)	0.000
KCNJ2	SDC3	psi-mi:“MI:0915”(physical association)	0.000
KCNJ2	KCNJ18	psi-mi:“MI:0915”(physical association)	0.000
KCNJ2	ARL13B	psi-mi:“MI:0915”(physical association)	0.000

BioGRID (36): KCNJ2 (Two-hybrid), KCNJ2 (Affinity Capture-Western), KCNJ2 (Two-hybrid), STK38 (Affinity Capture-Western), KCNJ2 (Affinity Capture-MS), USP15 (Affinity Capture-MS), RNPEP (Affinity Capture-MS), EPPK1 (Affinity Capture-MS), HSPA9 (Affinity Capture-MS), GRINA (Affinity Capture-MS), NONO (Affinity Capture-MS), FLG2 (Affinity Capture-MS), UBE4A (Affinity Capture-MS), MAN1B1 (Affinity Capture-MS), C3 (Affinity Capture-MS)

ESM2 similar proteins: B7U540, E1BNE9, F1MYR9, F1NHE9, O02670, O02822, O18839, O19182, O70617, P35561, P48050, P48051, P48542, P48543, P48544, P48545, P48548, P48549, P48550, P49656, P49658, P52185, P52186, P52187, P52188, P52189, P52190, P52191, P52192, P63250, P63251, P63252, P63253, P70673, P97794, Q14500, Q14654, Q15842, Q4TZY1, Q5NVJ6

Diamond homologs: B7U540, E1BN00, E1BNE9, F1MYR9, F1NHE9, O02670, O02822, O18839, O19182, O60928, O70339, O70596, O70617, O88335, O88932, P35560, P35561, P48048, P48050, P48051, P48542, P48543, P48544, P48545, P48548, P48549, P48550, P49655, P49656, P49658, P52185, P52186, P52187, P52188, P52189, P52190, P52191, P52192, P63250, P63251

SIGNOR signaling

2 interactions.

A	Effect	B	Mechanism
PKA	“down-regulates activity”	KCNJ2	phosphorylation
PRKACA	“down-regulates activity”	KCNJ2	phosphorylation

Disease & clinical

Clinical variants and AI predictions

ClinVar

684 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	42
Likely pathogenic	19
Uncertain significance	328
Likely benign	156
Benign	26

Top pathogenic / likely-pathogenic (30)

Variant ID	HGVS	Classification
1480008	NM_000891.3(KCNJ2):c.636G>A (p.Trp212Ter)	Pathogenic
2020840	NM_000891.3(KCNJ2):c.224C>A (p.Thr75Lys)	Pathogenic
2036326	NM_000891.3(KCNJ2):c.232G>A (p.Asp78Asn)	Pathogenic
234729	NM_000891.3(KCNJ2):c.902T>G (p.Met301Arg)	Pathogenic
2682647	NM_000891.3(KCNJ2):c.902T>A (p.Met301Lys)	Pathogenic
30119	NM_000891.3(KCNJ2):c.161G>T (p.Cys54Phe)	Pathogenic
30120	NM_000891.3(KCNJ2):c.913A>C (p.Thr305Pro)	Pathogenic
3220886	NM_000891.3(KCNJ2):c.269T>G (p.Leu90Arg)	Pathogenic
3243057	NC_000017.10:g.(?68171181)(70120528_?)del	Pathogenic
3243061	NC_000017.10:g.(?68166871)(68171602_?)del	Pathogenic
3755832	NM_000891.3(KCNJ2):c.1044C>G (p.Tyr348Ter)	Pathogenic
403974	NM_000891.3(KCNJ2):c.682C>T (p.Arg228Ter)	Pathogenic
463523	NM_000891.3(KCNJ2):c.715G>T (p.Glu239Ter)	Pathogenic
4784294	NM_000891.3(KCNJ2):c.351del (p.Glu118fs)	Pathogenic
569363	NM_000891.3(KCNJ2):c.1102del (p.Leu368fs)	Pathogenic
638351	NM_000891.3(KCNJ2):c.1177G>T (p.Gly393Ter)	Pathogenic
67566	NM_000891.3(KCNJ2):c.232G>T (p.Asp78Tyr)	Pathogenic
67567	NM_000891.3(KCNJ2):c.233A>G (p.Asp78Gly)	Pathogenic
67568	NM_000891.3(KCNJ2):c.244C>T (p.Arg82Trp)	Pathogenic
67569	NM_000891.3(KCNJ2):c.245G>A (p.Arg82Gln)	Pathogenic
67572	NM_000891.3(KCNJ2):c.407C>T (p.Ser136Phe)	Pathogenic
67573	NM_000891.3(KCNJ2):c.430G>A (p.Gly144Ser)	Pathogenic
67574	NM_000891.3(KCNJ2):c.431G>A (p.Gly144Asp)	Pathogenic
67575	NM_000891.3(KCNJ2):c.431G>C (p.Gly144Ala)	Pathogenic
67581	NM_000891.3(KCNJ2):c.574A>G (p.Thr192Ala)	Pathogenic
67583	NM_000891.3(KCNJ2):c.644G>A (p.Gly215Asp)	Pathogenic
67585	NM_000891.3(KCNJ2):c.653G>A (p.Arg218Gln)	Pathogenic
67587	NM_000891.3(KCNJ2):c.779G>C (p.Arg260Pro)	Pathogenic
67588	NM_000891.3(KCNJ2):c.899G>A (p.Gly300Asp)	Pathogenic
67591	NM_000891.3(KCNJ2):c.913A>G (p.Thr305Ala)	Pathogenic

SpliceAI

182 predictions. Top by Δscore:

Variant	Effect	Δscore
17:70168853:GCCTT:G	donor_gain	1.0000
17:70168858:G:GG	donor_gain	1.0000
17:70168854:CCTT:C	donor_gain	0.9900
17:70168856:TT:T	donor_gain	0.9900
17:70174822:A:AG	acceptor_gain	0.9900
17:70174823:G:GG	acceptor_gain	0.9900
17:70174823:GGAC:G	acceptor_gain	0.9900
17:70168855:CTTG:C	donor_loss	0.9800
17:70168856:TTG:T	donor_loss	0.9800
17:70168857:TGTA:T	donor_loss	0.9800
17:70168858:G:A	donor_loss	0.9800
17:70168859:TAAG:T	donor_loss	0.9800
17:70168860:A:AG	donor_loss	0.9800
17:70168861:AGT:A	donor_loss	0.9800
17:70168862:G:GG	donor_gain	0.9800
17:70174818:TTGCA:T	acceptor_loss	0.9800
17:70174819:TGCAG:T	acceptor_loss	0.9800
17:70174820:GCAG:G	acceptor_loss	0.9800
17:70174821:CA:C	acceptor_loss	0.9800
17:70174822:A:C	acceptor_loss	0.9800
17:70174822:AG:A	acceptor_gain	0.9800
17:70174823:GG:G	acceptor_gain	0.9800
17:70168855:CTT:C	donor_gain	0.9700
17:70168861:A:AG	donor_gain	0.9700
17:70174823:GGACA:G	acceptor_gain	0.9700
17:70168862:G:C	donor_loss	0.9600
17:70171200:T:G	acceptor_gain	0.9500
17:70174823:GGA:G	acceptor_gain	0.9500
17:70169180:G:T	donor_gain	0.9400
17:70172500:G:GT	donor_gain	0.9400

AlphaMissense

2850 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
17:70175470:G:A	G144D	1.000
17:70175499:T:C	C154R	1.000
17:70175622:T:C	F195L	1.000
17:70175624:C:A	F195L	1.000
17:70175624:C:G	F195L	1.000
17:70175625:A:C	S196R	1.000
17:70175627:T:A	S196R	1.000
17:70175627:T:G	S196R	1.000
17:70175673:T:A	W212R	1.000
17:70175673:T:C	W212R	1.000
17:70175677:G:C	R213P	1.000
17:70175687:T:A	N216K	1.000
17:70175687:T:G	N216K	1.000
17:70175719:T:A	V227D	1.000
17:70175724:G:C	A229P	1.000
17:70175836:C:A	P266Q	1.000
17:70175868:A:C	S277R	1.000
17:70175870:T:A	S277R	1.000
17:70175870:T:G	S277R	1.000
17:70175932:T:C	L298P	1.000
17:70175937:G:C	G300R	1.000
17:70175980:C:T	S314F	1.000
17:70176003:T:A	W322R	1.000
17:70176003:T:C	W322R	1.000
17:70176013:G:C	R325P	1.000
17:70175193:G:C	G52R	0.999
17:70175194:G:A	G52D	0.999
17:70175194:G:T	G52V	0.999
17:70175250:G:C	D71H	0.999
17:70175256:T:C	F73L	0.999

dbSNP variants (sampled 300 via entrez): RS1000887150 (17:70171523 C>T), RS1001224023 (17:70178236 G>A), RS1001469425 (17:70172338 G>A,C,T), RS1001548469 (17:70174383 G>A), RS1001642010 (17:70174580 T>C), RS1001683280 (17:70169028 G>A,T), RS1002110965 (17:70168778 C>A,G,T), RS1002217943 (17:70170903 C>T), RS1002273974 (17:70177823 A>C), RS1002491471 (17:70179860 G>A,T), RS1002590495 (17:70170500 G>A), RS1002762249 (17:70179584 C>A), RS1003171539 (17:70172244 C>T), RS1003228184 (17:70179127 G>A), RS1003760809 (17:70170149 A>G)

Disease associations

OMIM: gene MIM:600681 | disease phenotypes: MIM:170390, MIM:609622, MIM:192500, MIM:613980, MIM:114290, MIM:194200, MIM:609620, MIM:608583, MIM:601144

GenCC curated gene-disease

Disease	Classification	Inheritance
Andersen-Tawil syndrome	Definitive	Autosomal dominant
short QT syndrome type 3	Strong	Autosomal dominant
atrial fibrillation, familial, 9	Strong	Autosomal dominant
short QT syndrome	Moderate	Autosomal dominant
familial atrial fibrillation	Supportive	Autosomal dominant
catecholaminergic polymorphic ventricular tachycardia	Limited	Autosomal dominant
congenital heart disease	No Known Disease Relationship	Unknown

ClinGen Gene-Disease Validity (4)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

Disease	Classification	Inheritance
congenital heart disease	No Known Disease Relationship	UD
long QT syndrome	Limited	AD
short QT syndrome	Moderate	AD
catecholaminergic polymorphic ventricular tachycardia	Disputed	AD

Mondo (20): Andersen-Tawil syndrome (MONDO:0008222), short QT syndrome type 3 (MONDO:0012314), familial long QT syndrome (MONDO:0019171), atrial fibrillation, familial, 9 (MONDO:0013513), long QT syndrome (MONDO:0002442), ventricular fibrillation (MONDO:0000190), familial periodic paralysis (MONDO:0000995), atrial fibrillation (MONDO:0004981), campomelic dysplasia (MONDO:0007251), cardiac rhythm disease (MONDO:0007263), Wolff-Parkinson-White syndrome (MONDO:0008685), short QT syndrome (MONDO:0000453), ventricular tachycardia (MONDO:0005477), atrial fibrillation, familial, 1 (MONDO:0012066), short QT syndrome type 1 (MONDO:0012312)

Orphanet (9): Andersen-Tawil syndrome (Orphanet:37553), Congenital short QT syndrome (Orphanet:51083), Romano-Ward syndrome (Orphanet:101016), Congenital long QT syndrome (Orphanet:768), Genetic periodic paralysis (Orphanet:371433), Campomelic dysplasia (Orphanet:140), Rare hypertrophic cardiomyopathy (Orphanet:217569), Brugada syndrome (Orphanet:130), NON RARE IN EUROPE: Wolff-Parkinson-White syndrome (Orphanet:907)

HPO phenotypes

98 total (30 of 98 shown, HPO-id order):

HPO	Term
HP:0000006	Autosomal dominant inheritance
HP:0000089	Renal hypoplasia
HP:0000124	Renal tubular dysfunction
HP:0000164	Abnormality of the dentition
HP:0000175	Cleft palate
HP:0000218	High palate
HP:0000219	Thin upper lip vermilion
HP:0000252	Microcephaly
HP:0000272	Malar flattening
HP:0000316	Hypertelorism
HP:0000324	Facial asymmetry
HP:0000325	Triangular face
HP:0000327	Hypoplasia of the maxilla
HP:0000337	Broad forehead
HP:0000347	Micrognathia
HP:0000369	Low-set ears
HP:0000414	Bulbous nose
HP:0000431	Wide nasal bridge
HP:0000581	Blepharophimosis
HP:0000677	Oligodontia
HP:0000678	Dental crowding
HP:0000696	Delayed eruption of permanent teeth
HP:0000716	Depression
HP:0000836	Hyperthyroidism
HP:0000859	Increased circulating aldosterone concentration
HP:0001156	Brachydactyly
HP:0001250	Seizure
HP:0001279	Syncope
HP:0001324	Muscle weakness
HP:0001328	Specific learning disability

GWAS associations

120 associations (top):

Study	Trait	p-value
GCST000364_5	QT interval	6.000000e-12
GCST000609_5	Primary tooth development (time to first tooth eruption)	4.000000e-22
GCST000610_7	Primary tooth development (number of teeth)	1.000000e-14
GCST001410_1	Early cardiac repolarization	6.000000e-14
GCST001437_1	Thyrotoxic hypokalemic periodic paralysis	4.000000e-12
GCST001549_2	Formal thought disorder in schizophrenia	2.000000e-06
GCST001627_1	Thyrotoxic hypokalemic periodic paralysis	8.000000e-14
GCST001762_332	Obesity-related traits	9.000000e-06
GCST001762_630	Obesity-related traits	5.000000e-06
GCST001784_51	Pulmonary function (smoking interaction)	1.000000e-08
GCST001784_52	Pulmonary function (smoking interaction)	7.000000e-08
GCST001858_7	Refractive error	3.000000e-08
GCST002030_12	Primary tooth development (time to first tooth eruption)	8.000000e-34
GCST002031_10	Primary tooth development (number of teeth)	2.000000e-19
GCST002169_1	Adolescent idiopathic scoliosis (severe)	6.000000e-12
GCST002483_7	Lung function (forced vital capacity)	3.000000e-09
GCST002500_35	QT interval	6.000000e-11
GCST002500_36	QT interval	1.000000e-12
GCST002500_37	QT interval	2.000000e-25
GCST002500_38	QT interval	5.000000e-11
GCST002782_123	Waist-to-hip ratio adjusted for body mass index	4.000000e-09
GCST002782_124	Waist-to-hip ratio adjusted for body mass index	1.000000e-07
GCST002782_125	Waist-to-hip ratio adjusted for body mass index	1.000000e-09
GCST002782_126	Waist-to-hip ratio adjusted for body mass index	3.000000e-08
GCST003091_2	Depressive episodes in bipolar disorder	9.000000e-07
GCST003455_23	Spherical equivalent (joint analysis main effects and education interaction)	3.000000e-11
GCST003455_48	Spherical equivalent (joint analysis main effects and education interaction)	2.000000e-09
GCST003830_16	Response to bronchodilator in chronic obstructive pulmonary disease (change in FEV1)	2.000000e-07
GCST003830_34	Response to bronchodilator in chronic obstructive pulmonary disease (change in FEV1)	2.000000e-07
GCST003830_51	Response to bronchodilator in chronic obstructive pulmonary disease (change in FEV1)	3.000000e-07

EFO canonical traits (31, from GWAS)

EFO ID	Trait name
EFO:0004682	QT interval
EFO:0004644	TPE interval measurement
EFO:0004805	formal thought disorder
EFO:0004620	vitamin B12 measurement
EFO:0004611	low density lipoprotein cholesterol measurement
EFO:0003892	pulmonary function measurement
EFO:0004314	forced expiratory volume
EFO:0004312	vital capacity
EFO:0007788	BMI-adjusted waist-hip ratio
EFO:0007704	depressive episode measurement
EFO:0004784	self reported educational attainment
EFO:0005921	FEV change measurement
EFO:0008039	BMI-adjusted hip circumference
EFO:0004318	smoking behavior
EFO:0008002	physical activity measurement
EFO:0008111	diet measurement
EFO:0008007	age at assessment
EFO:0008343	sex interaction measurement
EFO:0004847	age at onset
EFO:0010078	dentures
EFO:0004344	birth weight
EFO:0008398	T wave morphology measurement
EFO:0010493	glycodeoxycholate measurement
EFO:0007805	HDL cholesterol change measurement
EFO:0004612	high density lipoprotein cholesterol measurement
EFO:0004530	triglyceride measurement
EFO:0007768	response to exercise
EFO:0004327	electrocardiography
EFO:0005665	white matter hyperintensity measurement
EFO:0004980	appendicular lean mass

MeSH disease descriptors (15)

Descriptor	Name	Tree numbers
D050030	Andersen Syndrome	C14.280.067.565.070; C14.280.123.625.070; C16.131.240.400.715.070; C23.550.073.547.070
D001281	Atrial Fibrillation	C14.280.067.198; C23.550.073.198
D053840	Brugada Syndrome	C14.280.067.322; C14.280.123.250; C16.320.100
D055036	Campomelic Dysplasia	C05.660.142; C16.131.621.142
D002312	Cardiomyopathy, Hypertrophic	C14.280.238.100; C14.280.484.048.750.070.160
D006330	Heart Defects, Congenital	C14.240.400; C14.280.400; C16.131.240.400
D008133	Long QT Syndrome	C14.280.067.565; C14.280.123.625; C16.131.240.400.715; C23.550.073.547
D010245	Paralyses, Familial Periodic	C05.651.701; C10.668.491.650; C16.320.565.618.711; C18.452.648.618.711
D017180	Tachycardia, Ventricular	C14.280.067.845.940; C14.280.123.875.940; C23.550.073.845.940
D014693	Ventricular Fibrillation	C14.280.067.922; C23.550.073.922
D014927	Wolff-Parkinson-White Syndrome	C14.280.067.780.977; C14.280.123.750.977; C16.131.240.400.980
C538261	Atrial fibrillation, familial 1 (supp.)
C566506	Short QT Syndrome 1 (supp.)
C566504	Short QT Syndrome 3 (supp.)
C580439	Short Qt Syndrome (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (1): CHEMBL1914276 (SINGLE PROTEIN)

PharmGKB: 1 entry (VIP=true, CPIC=false)

GtoPdb / IUPHAR curated pharmacology

(IUPHAR/BPS Guide to Pharmacology — expert-curated)

Target class: vgic — Inwardly rectifying potassium channels (K_IR)

Most potent curated ligand interactions (6 total), top 6:

Ligand	Action	Affinity	Parameter
spermine	Antagonist	9.1	pKd
spermidine	Antagonist	8.1	pKd
Ba2+	Antagonist	5.6	pKd
putrescine	Antagonist	5.1	pKd
Mg2+	Antagonist	4.8	pKd
Cs+	Antagonist	4.0	pKd

CTD chemical–gene interactions

73 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Doxorubicin	affects expression, increases expression	4
Valproic Acid	increases expression, affects cotreatment, decreases expression	4
Barium	decreases reaction, increases transport, decreases activity	3
Potassium	decreases reaction, increases transport	3
Tretinoin	affects expression, decreases expression, increases expression	3
Particulate Matter	decreases expression, increases abundance, affects cotreatment	3
bisphenol A	increases expression, increases methylation	2
sodium arsenite	increases expression	2
nickel sulfate	decreases expression	2
Benzo(a)pyrene	increases expression, increases methylation	2
Daunorubicin	increases expression	2
Estradiol	affects cotreatment, decreases expression, increases expression	2
Mitoxantrone	increases expression	2
Phenylmercuric Acetate	affects cotreatment, increases expression	2
Tobacco Smoke Pollution	decreases expression	2
Asbestos, Crocidolite	increases expression, decreases expression	2
3-((6-(2-methoxyphenyl)pyrimidin-4-yl)amino)phenyl)methane sulfonamide	decreases expression	1
TL8-506	affects cotreatment, increases expression	1
methylmercuric chloride	increases expression	1
triphenyl phosphate	affects expression	1
2-methyl-4-isothiazolin-3-one	increases expression	1
trichostatin A	decreases expression, increases expression	1
3,4-dichloroaniline	decreases expression	1
sulforaphane	increases expression	1
tobacco tar	decreases expression, decreases reaction	1
diallyl disulfide	decreases expression, decreases reaction	1
15-acetyldeoxynivalenol	increases expression	1
N-(2-(4-bromocinnamylamino)ethyl)-5-isoquinolinesulfonamide	decreases expression, decreases reaction	1
2-palmitoylglycerol	increases expression	1
4-(5-benzo(1,3)dioxol-5-yl-4-pyridin-2-yl-1H-imidazol-2-yl)benzamide	affects cotreatment, increases expression, decreases expression	1

ChEMBL screening assays

31 unique, capped per target: 23 binding, 8 admet

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL1918787	Binding	Inhibition of Kir2.1 at 10 uM after 5 mins by patch clamp assay	Optimization of propafenone analogues as antimalarial leads. — J Med Chem
CHEMBL4039285	ADMET	Inhibition of human Kir2.1 expressed in CHO cells by automated patch clamp assay	Discovery of N-(5-Fluoropyridin-2-yl)-6-methyl-4-(pyrimidin-5-yloxy)picolinamide (VU0424238): A Novel Negative Allosteric Modulator of Metabotropic Glutamate Receptor Subtype 5 Selected for Clinical Evaluation. — J Med Chem

Cellosaurus cell lines

2 cell lines: 1 spontaneously immortalized cell line, 1 transformed cell line

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_C0XW	B’SYS CHO Kir2.1	Spontaneously immortalized cell line	Female
CVCL_D1JS	PrecisION hKir2.1-HEK	Transformed cell line	Female

Clinical trials (associated diseases)

527 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT00668824	PHASE4	UNKNOWN	Improved Diagnosis of Congenital Heart Disease by Magnetic Resonance Imaging Using Vasovist
NCT01368705	PHASE4	COMPLETED	Nitrogen Balance in Infants After Post Cardiothoracic Surgery
NCT01619982	PHASE4	COMPLETED	Pre-operative Prophylaxis With Vancomycin and Cefazolin in Pediatric Cardiovascular Surgery Patients
NCT02122679	PHASE4	WITHDRAWN	Tranexamic Acid Effect on Platelet Aggregation Following Infant Cardiopulmonary Bypass
NCT02527811	PHASE4	UNKNOWN	Ulinastatin Injection in in Pediatric Patients Undergoing Open Heart Surgery
NCT03014700	PHASE4	COMPLETED	Fibrinogen Concentrate vs Cryoprecipitate
NCT03408340	PHASE4	TERMINATED	Paravertebral Nerve Blocks in Neonates
NCT03630796	PHASE4	UNKNOWN	Effect of Sevoflurane in Postoperative Troponin I Levels in Children Undergoing Congenital Heart Defects Surgery
NCT03667703	PHASE4	COMPLETED	Stress Ulcer Prophylaxis Versus Placebo in Critically Ill Infants With Congenital Heart Disease
NCT04453761	PHASE4	UNKNOWN	Thiamine Influenced on Substrate Energy Effectiveness in Indonesian Children Undergoing Cardiopulmonary Bypass
NCT06668389	PHASE4	RECRUITING	Sodium-Glucose Cotransporter 2 Inhibitors for Repaired Tetralogy of Fallot Patients for Enhancement of Cardio-Pulmonary Status Trial
NCT07499154	PHASE4	NOT_YET_RECRUITING	Perioperative Lidocaine for Lung Protection in Infants Undergoing Cardiac Surgery
NCT02513940	PHASE4	COMPLETED	Influence of Testosterone Administration on Drug-Induced QT Interval Prolongation and Torsades de Pointes
NCT03834883	PHASE4	COMPLETED	Reducing the Risk of Drug-Induced QT Interval Lengthening in Women
NCT04169100	PHASE4	UNKNOWN	Novel Form of Acquired Long QT Syndrome
NCT04675788	PHASE4	COMPLETED	Novel Approaches for Minimizing Drug-Induced QT Interval Lengthening
NCT00147277	PHASE4	COMPLETED	ADVANCE-D: Antitachycardia Pacing (ATP) Delivery for Painless Implantable Cardioverter Defibrillator (ICD) Therapy
NCT00147290	PHASE4	COMPLETED	ADVANCE CRT - D: Antitachycardia Pacing (ATP) Delivery for Painless Implantable Cardioverter Defibrillator (ICD) Therapy
NCT00180427	PHASE4	COMPLETED	VERRARI - Are Ventricular Arrhythmic Episodes Reduced by Rate Response in ICDs?
NCT00401466	PHASE4	COMPLETED	Remote Follow-up of Patients Receiving Implantable Cardioverter Defibrillator for Prophylactic Therapy
NCT00538356	PHASE4	COMPLETED	Influence of Home Monitoring on the Clinical Status of Heart Failure Patients With an Impaired Left Ventricular Function
NCT00787800	PHASE4	COMPLETED	The Use of Dual Chamber ICD With Special Programmed Features to Lower the Risk of Inappropriate Shock
NCT03826524	PHASE4	RECRUITING	Epinephrine Dose: Optimal Versus Standard Evaluation Trial
NCT03855826	PHASE4	UNKNOWN	Evaluation of the Efficacy and Safety of Nifekalant Hydrochloride (NIF) Injection.
NCT00032591	PHASE4	COMPLETED	The Home INR Study
NCT00127712	PHASE4	COMPLETED	Prevention of Atrial Fibrillation Following Noncardiac Thoracic Surgery
NCT00157781	PHASE4	COMPLETED	LEAF - Low Energy In Atrial Fibrillation
NCT00170313	PHASE4	TERMINATED	CORE: Study to Evaluate the Conducted AF-Response-Algorithm in Patients Suffering From Heart Failure and Atrial Fibrillation
NCT00189319	PHASE4	COMPLETED	To Evaluate the Impact of Oral Flecainide on Quality of Life in Patients With Paroxysmal Atrial Fibrillation
NCT00196144	PHASE4	COMPLETED	FFS - Far Field Sensing Test Study in Cardiac Dual Chamber Pacemakers
NCT00196157	PHASE4	UNKNOWN	Line Versus Spot Ablation in Persistent Atrial Fibrillation
NCT00196183	PHASE4	COMPLETED	Trigger- vs. Substrate-Ablation for Paroxysmal Atrial Fibrillation
NCT00196209	PHASE4	UNKNOWN	Cardioversion vs. Catheter Ablation for Persistent Atrial Fibrillation
NCT00227344	PHASE4	TERMINATED	CACAF2 Study: Catheter Ablation for Cure of Atrial Fibrillation
NCT00232219	PHASE4	COMPLETED	Use of Fish Oils to Reduce Recurrence of Atrial Fibrillation Following DC Cardioversion
NCT00232232	PHASE4	COMPLETED	Use of Fish Oils to Prevent Atrial Mechanical Stunning and Atrial Remodeling Due to Atrial Arrhythmia
NCT00232245	PHASE4	COMPLETED	Use of Fish Oils to Reduce the Frequency and Duration of Episodes of Atrial Fibrillation in Patients With Paroxysmal Atrial Fibrillation.
NCT00239226	PHASE4	COMPLETED	Electrophysiologically Guided PAcing Site Selection Study
NCT00247780	PHASE4	COMPLETED	Cavotricuspid Isthmusblock and Circumferential Pulmonary Vein Isolation in Patients With Atrial Fibrillation
NCT00256152	PHASE4	COMPLETED	Asymptomatic Atrial Fibrillation and Stroke Evaluation in Pacemaker Patients and the Atrial Fibrillation Reduction Atrial Pacing Trial