Description
Description: This assay employs a two-site sandwich ELISA to quantitate KCNQ4 in samples. An antibody specific for KCNQ4 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any KCNQ4 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for KCNQ4 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of KCNQ4 bound in the initial step. The color development is stopped and the intensity of the color is measured.
Overview: KCNQ4 forms a potassium channel that is thought to play a critical role in the regulation of neuronal excitability, particularly in sensory cells of the cochlea. The current generated by this channel is inhibited by M1 muscarinic acetylcholine receptors and activated by retigabine, a novel anti-convulsant drug.
The encoded protein can form a homomultimeric potassium channel or possibly a heteromultimeric channel in association with the protein encoded by the KCNQ3 gene. Defects in this gene are a cause of nonsyndromic sensorineural deafness type 2 (DFNA2), an autosomal dominant form of progressive hearing loss. Two transcript variants encoding different isoforms have been found for this gene