KCNH2, Polyclonal Antibody

ERG1; HERG; LQT2; SQT1; HERG1; Kv11.1

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total KCNH2 protein.

Antigen affinity purification.

Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.

0.2 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-KCNH2 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.

This gene encodes a voltage-activated potassium channel belonging to the eag family. It shares sequence similarity with the Drosophila ether-a-go-go (eag) gene. Mutations in this gene can cause long QT syndrome type 2 (LQT2). Transcript variants encoding distinct isoforms have been identified. Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1.

Total protein Ab

Western Blot (WB)

Western blotting: 1:500-1:2000

Gel: 8%SDS-PAGE Lysates (from left to right): Mouse brain tissue Amount of lysate: 30ug per lane Primary antibody: 1/100 dilution Secondary antibody dilution: 1/8000 Exposure time: 5 minutes

115,636 Da

potassium channel, voltage gated eag related subfamily H, member 2

potassium voltage-gated channel subfamily H member 2

Potassium voltage-gated channel subfamily H member 2

ERG; ERG1; HERG; ERG-1; Eag-related protein 1; Ether-a-go-go-related protein 1; H-ERG; hERG-1; hERG1  [Similar Products]

KCNH2_HUMAN

This gene encodes a voltage-activated potassium channel belonging to the eag family. It shares sequence similarity with the Drosophila ether-a-go-go (eag) gene. Mutations in this gene can cause long QT syndrome type 2 (LQT2). Transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Jul 2008]

Kv11.1: the ether-a-go-go related gene is a pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel is associated with cardiac arrhythmias and rhythmic excitability of the pituitary. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). The potassium channel is probably composed of a homo- or heterotetrameric complex of pore-forming alpha subunits that can associate with modulating beta subunits. Heteromultimer with Kv11.2 and Kv11.3. Interacts with ALG10B. Heteromultimer with KCNE1 and KCNE2. Defects in Kv11.1 are the cause of long QT syndrome type 2 (LQT2), a heart disorder 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. Deafness is often associated with LQT2. Defects in Kv11.1 are the cause of short QT syndrome type 1 (SQT1), a heart disorder 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. Four isoforms of the human protein are produced by alternative splicing. Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1.

Protein type: Motility/polarity/chemotaxis; Channel, potassium; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 7q36.1

Cellular Component: voltage-gated potassium channel complex; cell surface; cytoplasm; plasma membrane; nuclear envelope

Molecular Function: identical protein binding; protein binding; voltage-gated potassium channel activity; protein homodimerization activity; ubiquitin protein ligase binding; delayed rectifier potassium channel activity; inward rectifier potassium channel activity; two-component sensor activity

Biological Process: synaptic transmission; regulation of membrane potential; two-component signal transduction system (phosphorelay); regulation of the rate of heart contraction by hormone; potassium ion homeostasis; cardiac muscle contraction

Disease: Long Qt Syndrome 2; Short Qt Syndrome 1

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