Potassium Inwardly-Rectifying Channel, Subfamily J, Member 2 (KCNJ2) (Cytoplasmic Domain) antibody

Antigen: Potassium Inwardly-Rectifying Channel, Subfamily J, Member 2 (KCNJ2)

Synonyms: IRK1, LQT7, SQT3, HHIRK1, KIR2.1, HHBIRK1, Kcnf1, Kir2.1

Binding Site: Cytoplasmic Domain

Clonality: Polyclonal

Host: Rabbit

Reactivity: Please enquire

Application: Immunohistochemistry (IHC), Western Blotting (WB)

Catalog no.: ABIN350393

Additional Information

Alternative name: KCNJ2 (HIRK1, IRK1, Kir2.1)

UniProt: P35561

Immunogen: A synthetic peptide from the cytoplasmic domain of mouse KCNJ2 (HIRK1, IRK1, Kir2.1) conjugated to an immunogenic carrier protein was used as the immunogen. The antigen is homologous in rat and human.

Format: Lyophilized

Description: FUNCTION: Probably participates in establishing action potential waveform and excitability of neuronal and muscle tissues. 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. Tissue specificity: Heart, brain, placenta, lung, skeletal muscle, and kidney. Diffusely distributed throughout the brain. Subcellular location: Membrane, Multi-pass membrane protein. Involvement in disease: Defects in KCNJ2 are the cause of long QT syndrome type 7 (LQT7); also called Andersen syndrome or Andersen cardiodysrhythmic periodic paralysis. Long QT syndromes are heart disorders characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to excercise or emotional stress. LQT7 manifests itself as a clinical triad consisting of potassium-sensitive periodic paralysis, ventricular ectopy and dysmorphic features. Defects in KCNJ2 are the cause of short QT syndrome type 3 (SQT3). Short QT syndromes are heart disorders characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death. SQT3 has a unique ECG phenotype characterized by asymmetrical T waves. Also known as: Inward rectifier potassium channel 2, Potassium channel, inwardly rectifying subfamily J member 2, Inward rectifier K(+) channel Kir2.1, Cardiac inward rectifier potassium channel, IRK1.

Specificity: Appears to be specific for KCNJ2.

Application Details

Application Notes: IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Purity: whole serum

Storage: Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

Restrictions: For Research Use only

Publications

Wood, Tsai, Lee et al.: "Cloning and functional expression of a human gene, hIRK1, encoding the heart inward rectifier K+-channel." in: Gene, Vol. 163, Issue 2, pp. 313-7, 1995 (PubMed).

Raab-Graham, Radeke, Vandenberg: "Molecular cloning and expression of a human heart inward rectifier potassium channel." in: Neuroreport, Vol. 5, Issue 18, pp. 2501-5, 1995 (PubMed).

Tare, Prestwich, Gordienko et al.: "Inwardly rectifying whole cell potassium current in human blood eosinophils." in: The Journal of physiology, Vol. 506 ( Pt 2), pp. 303-18, 1998 (PubMed).

Derst, Karschin, Wischmeyer et al.: "Genetic and functional linkage of Kir5.1 and Kir2.1 channel subunits." in: FEBS letters, Vol. 491, Issue 3, pp. 305-11, 2001 (PubMed).

Plaster, Tawil, Tristani-Firouzi et al.: "Mutations in Kir2.1 cause the developmental and episodic electrical phenotypes of Andersen's syndrome." in: Cell, Vol. 105, Issue 4, pp. 511-9, 2001 (PubMed).

Priori, Pandit, Rivolta et al.: "A novel form of short QT syndrome (SQT3) is caused by a mutation in the KCNJ2 gene." in: Circulation research, Vol. 96, Issue 7, pp. 800-7, 2005 (PubMed).