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

Chromosome: 1; NC_000001.10 (41249684..41306124). Location: 1p34

E Coli or Yeast or Baculovirus or Mammalian Cell

>=90% (lot specific)

Liquid containing glycerol

This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.

Sterile filter available upon request.

Low endotoxin available upon request.

Store at -20 degree C. For extended storage, store at -20 or -80 degree C.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of KCNQ4 recombinant protein 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.

KCNQ4

77,101 Da[Similar Products]

potassium voltage-gated channel, KQT-like subfamily, member 4

potassium voltage-gated channel subfamily KQT member 4; potassium channel KQT-like 4; potassium channel subunit alpha KvLQT4

Potassium voltage-gated channel subfamily KQT member 4

KCNQ4_HUMAN

The protein encoded by this gene 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. [provided by RefSeq, Jul 2008]

Function: Probably important in the regulation of neuronal excitability. May underlie a potassium current involved in regulating the excitability of sensory cells of the cochlea. KCNQ4 channels are blocked by linopirdin, XE991 and bepridil, whereas clofilium is without significant effect. Muscarinic agonist oxotremorine-M strongly suppress KCNQ4 current in CHO cells in which cloned KCNQ4 channels were coexpressed with M1 muscarinic receptors. Ref.4

Subunit structure: Homotetramer. May form heteromultimers with KCNQ3. Ref.5

Subcellular location: Basal cell membrane; Multi-pass membrane protein. Note: Situated at the basal membrane of cochlear outer hair cells

By similarity.

Tissue specificity: Expressed in the outer, but not the inner, sensory hair cells of the cochlea. Slightly expressed in heart, brain and skeletal muscle.

Domain: The segment S4 is probably the voltage-sensor and is characterized by a series of positively charged amino acids at every third position

By similarity. Ref.5The A-domain tail carries the major determinants of channel assembly specificity. Its coiled-coil region is Four-stranded. Ref.5

Involvement in disease: Defects in KCNQ4 are the cause of deafness autosomal dominant type 2A (DFNA2A) [

MIM:600101]. DFNA2A is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Ref.1 Ref.6 Ref.7 Ref.8 Ref.9

Miscellaneous: Mutagenesis experiments were carried out by expressing in Xenopus oocytes KCNQ4 mutants either individually (homomultimers) or in combination with wild-type KCNQ4 (mut/wt homomultimers) in a ratio of 1:1, to mimic the situation in a heterozygous DFNA2 patient.

Sequence similarities: Belongs to the potassium channel family. KQT (TC 1.A.1.15) subfamily. Kv7.4/KCNQ4 sub-subfamily. [View classification]

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