KCNH2 / HERG, Polyclonal Antibody

Anti-KCNH2 / HERG Antibody (Internal) IHC-plus; KCNH2; Eag homolog; HERG-1; ERG1; HERG; KV11.1; M-erg1; H-ERG; HERG1; SQT1; LQT2; Merg1; Eag-related protein 1; ERG-1; Human KCNH2; HERG

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

Polyclonal

Rabbit

Human KCNH2 / Kv11.1. BLAST analysis of the peptide immunogen showed no homology with other human proteins.

Immunoaffinity Purified

PBS, 0.1% sodium azide.

0.9 mg/ml (lot specific)

Long term: -70 degree C; Short term: +4 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.

KCNH2 encodes a human homologue of the Drosophila melanogaster eag gene, a potassium channel protein. Along with five other human genes, mutations in KCNH2 that result in loss of function or dominant negative function cause long QT syndrome.

Subfamily:-Potassium-channel---Eag-related">Family: Ion Channel
Subfamily: Potassium channel - Eag-related

Immunohistochemistry (IHC - Paraffin)

IHC-P (5 ug/ml)

Anti-KCNH2 / Kv11.1 antibody IHC of human brain, cortex. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.

115,636 Da

potassium voltage-gated channel, subfamily H (eag-related), member 2

potassium voltage-gated channel subfamily H member 2; eag homolog; eag-related protein 1; ether-a-go-go-related protein 1; ether-a-go-go-related gene potassium channel 1; voltage-gated potassium channel subunit Kv11.1; ether-a-go-go-related potassium channel protein

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; Membrane protein, integral; Channel, potassium; 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; voltage-gated potassium channel activity; protein binding; protein homodimerization activity; delayed rectifier potassium channel activity; ubiquitin protein ligase binding; inward rectifier potassium channel activity; two-component sensor activity

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

Disease: Long Qt Syndrome 2; Short Qt Syndrome 1

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.