Code
OSK00013W
$240USD
ID Tag
Rb863-290309-WS
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.
Accession
Also known
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
Target
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.
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.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Raab-Graham K.F, et al. NeuroReport 5:2501-2505(1994)
6. Priori S.G, et al. Circ. Res. 96:800-807(2005)
Limitation
For research use only
Related
products
Code
OSK00013W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
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.
Specificity
Specific for KCNJ2.
Spcs X-react.
Human, rat, mouse. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.