KCNJ2

Official Full Name

potassium inwardly rectifying channel subfamily J member 2

Organism

Homo sapiens

Gene ID

3759

Background

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. [provided by RefSeq, Jul 2008]

Synonyms

IRK1; LQT7; SQT3; ATFB9; HHIRK1; KIR2.1; HHBIRK1

Cat.No.	Product Name	Price
CSC-RI0066	Human CACNA1C/NEUROD1/CACNA2D1/KCNJ2 Stable Cell Line-HEK293	Inquiry
CSC-RI0070	Human CACNA1D/CACNB3/CACNA2D1 + KCNJ2(tetracycline-inducible) Stable Cell Line-HEK293	Inquiry
CSC-RI0072	Human CACNA1A/AP4B1/CACNA2D1/KCNJ2 Stable Cell Line-HEK293	Inquiry
CSC-RI0074	Human CACNA1B/BHLHE22/CACNA2D1/KCNJ2 Stable Cell Line-HEK293	Inquiry
CSC-RI0080	Human CACNA1H/CACNB2/CACNA2D1/KCNJ2 Stable Cell Line-HEK293	Inquiry
CSC-RI0115	Human KCNJ2 Stable Cell Line-HEK293	Inquiry
CSC-RI0116	Human KCNJ2 Stable Cell Line-CHO	Inquiry
CSC-DC007968	Panoply™ Human KCNJ2 Knockdown Stable Cell Line	Inquiry
CSC-SC007968	Panoply™ Human KCNJ2 Over-expressing Stable Cell Line	Inquiry

Cat.No.	Product Name	Price
AD08425Z	Human KCNJ2 adenoviral particles	Inquiry
LV15994L	human KCNJ2 (NM_000891) lentivirus particles	Inquiry

Cat.No.	Product Name	Price
SHH323279	shRNA set against Mouse KCNJ2 (NM_008425.4)	Inquiry
SHH323283	shRNA set against Rat KCNJ2 (NM_017296.1)	Inquiry
SHL189804	shRNA set against Human KCNJ2(NM_000891.2)	Inquiry
SHW005865	shRNA set against Chicken KCNJ2 (NM_205370)	Inquiry
SHH323275	shRNA set against Human KCNJ2 (NM_000891.2)	Inquiry
SHL189876	shRNA set against Rat Kcnj2(NM_017296.1)	Inquiry
SHW014197	shRNA set against Danio rerio KCNJ2A (NM_001278802)	Inquiry

Cat.No.	Product Name	Price
CDCR378397	Rat Kcnj2 ORF Clone(NM_017296.1)	Inquiry
CDFH009651	Human KCNJ2 cDNA Clone(NM_000891.2)	Inquiry
CDFR011343	Rat Kcnj2 cDNA Clone(NM_017296.1)	Inquiry
MiUTR1H-05137	KCNJ2 miRNA 3'UTR clone	Inquiry
MiUTR1R-02822	KCNJ2 miRNA 3'UTR clone	Inquiry
CDCB167340	Chicken KCNJ2 ORF Clone (NM_205370)	Inquiry
CDCB175672	Danio rerio KCNJ2A ORF Clone (NM_001278802)	Inquiry
CDCB180461	Rabbit KCNJ2 ORF clone (NM_001082198.1)	Inquiry
CDCR245297	Mouse Kcnj2 ORF Clone(NM_008425.4)	Inquiry
CDCR337937	Human KCNJ2 ORF Clone(NM_000891.2)	Inquiry

Detailed Information

Recent Research

KCNJ2, encodes Kir2.1, is a member of the classical inwardly rectifying potassium channel family (Kir2 subfamily). It is a 427 amino acid, double-channel transmembrane protein, located at both ends in the cytoplasm. It conducts a strong inward rectifier K+ current in a wide range of tissues and cell types, including neurons, skeletal muscle, cardiac myocytes, immune system, hippocampus, caudate, putamen and carcinoma cells. The KCNJ2 gene was first cloned by Kubo from a macrophage cell line. Similar to the other members of the Kir family, KCNJ2 is tetrameric, containing two transmembrane helix domains (M1 and M2), an ion-selective P-loop between M1 and M2, and cytoplasmic N-and C-terminal domains. Functionally, KCNJ2 plays a key role in maintaining the resting membrane potential and regulating cellular excitability in cardiac myocytes, skeletal muscle and neurons. Changes in the expression levels of K+ channels induced by aberrant KCNJ2 expression have substantial effects on cellular processes such as cell death, proliferation, apoptosis and adhesion, which are linked to a variety of cardiac and neurological disorders.

It is reported that KCNJ2 loss-of-function mutations result in four distinct arrhythmia syndromes, including Andersen-Tawil syndrome type 1 (ATS1, also denoted as long QT syndrome type 7 [LQT7]), short QT syndrome type 3(SQT), familial atrial fibrillation and CPVT3. ATS1 is a rare autosomal-dominant disorder. It is characterized by a phenotypic triad consisting of cardiac abnormalities (premature ventricular complexes, ventricular bigeminy, ventricular tachycardia and supraventricular, torsades de pointes, prominent electrocardiographic U waves and prolonged QT intervals) in addition to dysmorphic features and periodic paralysis.

In addition, gain-of-function KCNJ2 mutations cause atrial fibrillation (AF). Atrial fibrillation is the most common arrhythmia characterized by complex and irregular atrial electrical activity. One rare, genetic condition associated with increased risk of AF is the SQT, which is caused by mutations in genes involved in normal electrical function of the heart. SQT results in QT shortening and increased risk of sudden cardiac death.

References:

Anna B, et al. A Novel KCNJ2 Mutation Identified in an Autistic Proband Affects the Single Channel Properties of Kir2.1. Frontiers in Cellular Neuroscience, 2018, 12:76-.
Whittaker D G, et al. Atrial arrhythmogenicity of KCNJ2 mutations in short QT syndrome: Insights from virtual human atria. PLOS Computational Biology, 2017, 13(6):e1005593-.
Adams D S, et al. Bioelectric signalling via potassium channels: a mechanism for craniofacial dysmorphogenesis in KCNJ2-associated Andersen-Tawil Syndrome. The Journal of Physiology, 2016:n/a-n/a.
Liu H, et al. Upregulation of the inwardly rectifying potassium channel Kir2.1 (KCNJ2) modulates multidrug resistance of small-cell lung cancer under the regulation of miR-7 and the Ras/MAPK pathway. Molecular Cancer, 2015, 14(1):59.

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