KCNE1, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Purified by antigen-specific affinity chromatography.

Liquid

1ug/ul (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Small volumes of anti-KCNE1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

KCNE1 (Potassium voltage-gated channel subfamily E member 1) is an ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. KCNE1 modulates the gating kinetics and enhances stability of the channel complex. The KCNE1 assembled with KCNQ1/KVLQT1 is proposed to form the slowly activating delayed rectifier cardiac potassium (IKs) channel. The outward current reaches its steady state only after 50 seconds. The KCNE1 assembled with KCNH2/HERG may modulate the rapidly activating component of the delayed rectifying potassium current in heart (IKr). KCNE1 is associates with KCNQ1/KVLQT1 and KCNH2/HERG. Defects in KCNE1 are a cause of the autosomal recessive Jervell and Lange-Nielsen syndrome (JLNS). JLNS comprises profound congenital sensorineural deafness associated with syncopal episodes. These are caused by ventricular tachyarrhythmia secondary to abnormal repolarization, manifested by a prolonged QT interval on the electrocardiogram.

Western Blot (WB), ELISA (EIA)

14,675 Da

potassium voltage-gated channel subfamily E regulatory subunit 1

potassium voltage-gated channel subfamily E member 1

Potassium voltage-gated channel subfamily E member 1

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]

KCNE1: Ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. Modulates the gating kinetics and enhances stability of the channel complex. Assembled with KCNQ1/KVLQT1 is proposed to form the slowly activating delayed rectifier cardiac potassium (IKs) channel. The outward current reaches its steady state only after 50 seconds. Assembled with KCNH2/HERG may modulate the rapidly activating component of the delayed rectifying potassium current in heart (IKr). Associates with KCNQ1/KVLQT1 and KCNH2/HERG. Expressed in heart, lung, kidney, testis, ovaries, small intestine, peripheral blood leukocytes. Not detected in pancreas, spleen, prostate and colon. Restrictively localized in the apical membrane portion of epithelial cells. Belongs to the potassium channel KCNE family.

Protein type: Channel, potassium; Membrane protein, integral

Chromosomal Location of Human Ortholog: 21q22.12

Cellular Component: apical plasma membrane; cell surface; lysosome; plasma membrane; voltage-gated potassium channel complex; Z disc

Molecular Function: delayed rectifier potassium channel activity; potassium channel regulator activity; protein binding; telethonin binding; voltage-gated potassium channel activity

Biological Process: protein amino acid N-linked glycosylation; protein amino acid O-linked glycosylation; sensory perception of sound

Disease: Jervell And Lange-nielsen Syndrome 2; Long Qt Syndrome 5

Murai, T., et al, Biochem. Biophys. Res. Commun. 161 (1), 176-181 (1989)Chouabe, C., et al, EMBO J. 16 (17),5472-5479 (1997)McDonald, T.V., et al, Nature 388 (6639), 289-292 (1997)Abbott, G.W. and Goldstein, S.A., FASEB J. 16 (3), 390-400 (2002)Tesson, F., et al, J. Mol. Cell. Cardiol. 28 (9), 2051-2055 (1996)Tyson, J., et al, Hum. Mol. Genet. 6 (12), 2179-2185 (1997)Schulze-Bahr, E., et al, Nat. Genet. 17 (3), 267-268 (1997)Splawski, I., et al, Nat. Genet. 17 (3), 338-340 (1997)Duggal, P., et al, Circulation 97 (2), 142-146 (1998)Splawski, I., et al, Circulation 102 (10), 1178-1185 (2000)Schulze-Bahr, E., et al, J. Mol. Med. 79 (9),504-509 (2001)

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