KCNQ2, Monoclonal Antibody

BFNC; EBN1; ENB1; HNSPC; KCNA11; KQT like 2; KV7.2; KVEBN1. KvLQT2; voltage gated potassium channel subunit Kv7.2

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

Monoclonal

IgG1

S26A-23

Mouse

Protein G Purified

1mg/mL (lot specific)

-20 degree C

Small volumes of anti-KCNQ2 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.

Background Info: Detects ~95kDa

Scientific Background: Ion channels are integral membrane proteins that help establish and control the small voltage gradient across the plasma membrane of living cells by allowing the flow of ions down their electrochemical gradient (1). They are present in the membranes that surround all biological cells because their main function is to regulate the flow of ions across this membrane. Whereas some ion channels permit the passage of ions based on charge, others conduct based on a ionic species, such as sodium or potassium. Furthermore, in some ion channels, the passage is governed by a gate which is controlled by chemical or electrical signals, temperature, or mechanical forces. There are a few main classifications of gated ion channels. There are voltage- gated ion channels, ligand- gated, other gating systems and finally those that are classified differently, having more exotic characteristics. The first are voltage- gated ion channels which open and close in response to membrane potential. These are then separated into sodium, calcium, potassium, proton, transient receptor, and cyclic nucleotide-gated channels; each of which is responsible for a unique role. Ligand-gated ion channels are also known as ionotropic receptors, and they open in response to specific ligand molecules binding to the extracellular domain of the receptor protein. The other gated classifications include activation and inactivation by second messengers, inward-rectifier potassium channels, calcium-activated potassium channels, two-pore-domain potassium channels, light-gated channels, mechano-sensitive ion channels and cyclic nucleotide-gated channels. Finally, the other classifications are based on less normal characteristics such as two-pore channels, and transient receptor potential channels (2). Specifically, Kv7.2 (KvLQT2) is a potassium channel protein coded for by the gene KCNQ2. It is associated with benign familial neonatal convulsions (3).

Ion Channels, Neuroscience

Western Blot (WB), Immunoprecipitation (IP), Immunohistochemistry (IHC), Immunofluorescence (IF)

1-10ug/mL (WB), 0.1-1.0ug/mL (Perox) (IHC/ICC), 1.0-10ug/mL (IF)

Immunohistochemistry analysis using Mouse Anti-KCNQ2 Monoclonal Antibody, Clone S26A-23. Tissue: frozen brain section. Species: Mouse. Fixation: 10% Formalin Solution for 12-24 hours at RT. Primary Antibody: Mouse Anti-KCNQ2 Monoclonal Antibody at 1:1000 for 1 hour at RT. Secondary Antibody: HRP/DAB Detection System: Biotinylated Goat Anti-Mouse, Streptavidin Peroxidase, DAB Chromogen (brown) for 30 minutes at RT. Counterstain: Mayer Hematoxylin (purple/blue) nuclear stain at 250-500 ul for 5 minutes at RT.

Western Blot analysis of Human, hamster HEK and CHO cell lysates showing detection of KCNQ2 protein using Mouse Anti-KCNQ2 Monoclonal Antibody, Clone S26A-23. Load: 15 ug. Block: 1.5% BSA for 30 minutes at RT. Primary Antibody: Mouse Anti-KCNQ2 Monoclonal Antibody at 1:1000 for 2 hours at RT. Secondary Antibody: Sheep Anti-Mouse IgG: HRP for 1 hour at RT. KCNQ2 overexpressed.

Immunohistochemistry analysis using Mouse Anti-KCNQ2 Monoclonal Antibody, Clone S26A-23. Tissue: hippocampus. Species: Human. Fixation: Bouin's Fixative and paraffin-embedded. Primary Antibody: Mouse Anti-KCNQ2 Monoclonal Antibody at 1:1000 for 1 hour at RT. Secondary Antibody: FITC Goat Anti-Mouse (green) at 1:50 for 1 hour at RT.

44,261 Da[Similar Products]

potassium voltage-gated channel, KQT-like subfamily, member 2

potassium voltage-gated channel subfamily KQT member 2; KQT-like 2; voltage-gated potassium channel subunit Kv7.2; neuroblastoma-specific potassium channel protein; neuroblastoma-specific potassium channel subunit alpha KvLQT2

Potassium voltage-gated channel subfamily KQT member 2

KCNQ2_HUMAN

The M channel is a slowly activating and deactivating potassium channel that plays a critical role in the regulation of neuronal excitability. The M channel is formed by the association of the protein encoded by this gene and a related protein encoded by the KCNQ3 gene, both integral membrane proteins. M channel currents are inhibited by M1 muscarinic acetylcholine receptors and activated by retigabine, a novel anti-convulsant drug. Defects in this gene are a cause of benign familial neonatal convulsions type 1 (BFNC), also known as epilepsy, benign neonatal type 1 (EBN1). At least five transcript variants encoding five different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Kv7.2: Probably important in the regulation of neuronal excitability. Associates with KCNQ3 to form a potassium channel with essentially identical properties to the channel underlying the native M-current, a slowly activating and deactivating potassium conductance which plays a critical role in determining the subthreshold electrical excitability of neurons as well as the responsiveness to synaptic inputs. KCNQ2/KCNQ3 current is blocked by linopirdine and XE991, and activated by the anticonvulsant retigabine. Muscarinic agonist oxotremorine-M strongly suppress KCNQ2/KCNQ3 current in cells in which cloned KCNQ2/KCNQ3 channels were coexpressed with M1 muscarinic receptors. Defects in KCNQ2 are the cause of benign familial neonatal seizures type 1 (BFNS1). A disorder characterized by clusters of seizures occurring in the first days of life. Most patients have spontaneous remission by 12 months of age and show normal psychomotor development. Some rare cases manifest an atypical severe phenotype associated with epileptic encephalopathy and psychomotor retardation. The disorder is distinguished from benign familial infantile seizures by an earlier age at onset. In some patients, neonatal convulsions are followed later in life by myokymia, a benign condition characterized by spontaneous involuntary contractions of skeletal muscles fiber groups that can be observed as vermiform movement of the overlying skin. Electromyography typically shows continuous motor unit activity with spontaneous oligo- and multiplet- discharges of high intraburst frequency (myokymic discharges). Some patients may have isolated myokymia. Defects in KCNQ2 are the cause of epileptic encephalopathy early infantile type 7 (EIEE7). EIEE7 is an autosomal dominant seizure disorder characterized by infantile onset of refractory seizures with resultant delayed neurologic development and persistent neurologic abnormalities. Belongs to the potassium channel family. KQT (TC 1.A.1.15) subfamily. Kv7.2/KCNQ2 sub-subfamily. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Channel, potassium

Chromosomal Location of Human Ortholog: 20q13.3

Cellular Component: voltage-gated potassium channel complex; integral to membrane; plasma membrane

Molecular Function: voltage-gated potassium channel activity; potassium channel activity; delayed rectifier potassium channel activity; ankyrin binding

Biological Process: axon guidance; nervous system development; synaptic transmission; transmission of nerve impulse; potassium ion transport

Disease: Seizures, Benign Familial Neonatal, 1; Epileptic Encephalopathy, Early Infantile, 7

1. Hille B. (2001) Ion Channels of Excitable Membranes, 3rd Ed., Sinauer Associated Inc.: Sunderland, MA USA. 2. www.iochannels.org 3. Wuttke T.V., et al. (2008) J Physiol. 586(2): 545-555.

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