Home > Product > Antibody > Rabbit Anti-KCNQ4/FITC Conjugated antibody
DFNA 2; DFNA2; KCNQ 4; Kcnq4; KCNQ4_HUMAN; KQT like 4; KQT-like 4; KV7.4; Potassium channel KQT like 4; Potassium channel subunit alpha KvLQT4; Potassium voltage gated channel KQT like protein 4; Potassium voltage gated channel KQT like subfami
Cat:
SL16913R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Sheep,)
Immunogen:
KLH conjugated synthetic peptide derived from human KCNQ4
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
77kDa
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Unit:
Price: $
Product PDFs
Datasheet:


background:
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 muscarinnic receptors.

Subcellular Location:
Basal cell membrane. Situated at the basal membrane of cochlear outer hair cells.

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

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.

Similarity:
Belongs to the potassium channel family. KQT (TC 1.A.1.15) subfamily. Kv7.4/KCNQ4 sub-subfamily.

Database links:

Entrez Gene: 9132 Human

Entrez Gene: 60613 Mouse

Entrez Gene: 298496 Rat

Omim: 603537 Human

SwissProt: P56696 Human

SwissProt: Q9JK97 Mouse

SwissProt: Q9JK96 Rat

Unigene: 473058 Human

Unigene: 249977 Mouse

Unigene: 144875 Rat

Unigene: 225305 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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