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Rabbit Anti-KCNQ4/BF350 Conjugated antibody (bs-16913R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-16913R-BF350
英文名稱1 Rabbit Anti-KCNQ4/BF350 Conjugated antibody
中文名稱 BF350標記的電壓門控鉀通道亞基Kv7.4抗體
別    名 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 subfamily member 4; Potassium voltage gated channel subfamily KQT member 4; Potassium voltage-gated channel subfamily KQT member 4; Voltage gated potassium channel subunit Kv7.4; Voltage-gated potassium channel subunit Kv7.4.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 77kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KCNQ4
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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 antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 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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