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KCNE1 Rabbit pAb
KCNE1 Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application 
| WB, IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P15382 |
| Predicted | Rat, Pig, Dog, Human, Mouse, Horse, Sheep |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 14675 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human KCNE1 |
| Epitope Specificity | 41-129/129 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Membrane; Single-pass type I membrane protein. |
| DISEASE | Defects in KCNE1 are the cause of Jervell and Lange-Nielsen syndrome type 2 (JLNS2) [MIM:612347]. JLNS2 is an autosomal recessive disorder characterized by congenital deafness, prolongation of the QT interval, syncopal attacks due to ventricular arrhythmias, and a high risk of sudden death. Defects in KCNE1 are the cause of long QT syndrome type 5 (LQT5) [MIM:613695]. 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 exercise or emotional stress. KCNE1 mutants form channels that open slowly and close rapidly, thereby diminishing potassium currents. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The product of this gene belongs to the potassium channel KCNE family. Potassium ion channels are essential to many cellular functions and show a high degree of diversity, varying in their electrophysiologic and pharmacologic properties. This gene encodes a transmembrane protein known to associate with the product of the KVLQT1 gene to form the delayed rectifier potassium channel. Mutation in this gene are associated with both Jervell and Lange-Nielsen and Romano-Ward forms of long-QT syndrome. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008]. |
| Gene ID | 3753 |
|---|---|
| Other Names | Potassium voltage-gated channel subfamily E member 1, Delayed rectifier potassium channel subunit IsK, IKs producing slow voltage-gated potassium channel subunit beta Mink, Minimal potassium channel, MinK, KCNE1 (HGNC:6240) |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | KCNE1 (HGNC:6240) |
|---|---|
| Function | Ancillary protein that functions as a regulatory subunit of the voltage-gated potassium (Kv) channel complex composed of pore- forming and potassium-conducting alpha subunits and of regulatory beta subunits. KCNE1 beta subunit modulates the gating kinetics and enhances stability of the channel complex (PubMed:19219384, PubMed:20533308, PubMed:9230439). Alters the gating of the delayed rectifier Kv channel containing KCNB1 alpha subunit (PubMed:19219384). Associates with KCNQ1/KVLQT1 alpha subunit to form the slowly activating delayed rectifier cardiac potassium (IKs) channel responsible for ventricular muscle action potential repolarization (PubMed:20533308). The outward current reaches its steady state only after 50 seconds (Probable). Assembly with KCNH2/HERG alpha subunit Kv channel may regulate the rapidly activating component of the delayed rectifying potassium current (IKr) in heart (PubMed:9230439). |
| Cellular Location | Cell membrane; Single-pass type I membrane protein. Apical cell membrane {ECO:0000250|UniProtKB:P15383}. Membrane raft. Note=Colocalizes with KCNB1 at the plasma membrane (By similarity). Targets to the membrane raft when associated with KCNQ1 (PubMed:20533308). {ECO:0000250|UniProtKB:P15383, ECO:0000269|PubMed:20533308} |
| Tissue Location | Expressed in lung, kidney, testis, ovaries, small intestine, peripheral blood leukocytes. Expressed in the heart (PubMed:19219384). Not detected in pancreas, spleen, prostate and colon. Restrictively localized in the apical membrane portion of epithelial cells. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
REFERENCES
Murai T.,et al.Biochem. Biophys. Res. Commun. 161:176-181(1989).
Lai L.P.,et al.Gene 151:339-340(1994).
Rae J.L.,et al.Submitted (MAR-1999) to the EMBL/GenBank/DDBJ databases.
Chouabe C.,et al.EMBO J. 16:5472-5479(1997).
McDonald T.V.,et al.Nature 388:289-292(1997).
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Cat# AP52133
