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Rabbit Anti-KCNQ2/BF555 Conjugated antibody (bs-11728R-BF555)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11728R-BF555
英文名稱 Rabbit Anti-KCNQ2/BF555 Conjugated antibody
中文名稱 BF555標記的鉀離子通道蛋白家族KCNQ2抗體
別    名 BFNC; BFNS1; EBN 1; EBN; EBN1; EIEE7; ENB 1; ENB1; HNSPC; KCNA 11; KCNA11; KCNQ 2; Kcnq2; KCNQ2_HUMAN; KQT like 2; KQT-like 2; KV7.2; KVEBN 1; KVEBN1; KvLQT 2; KvLQT2; Neuroblastoma specific potassium channel alpha subunit KvLQT2; Neuroblastoma specific potassium channel protein; Neuroblastoma specific potassium channel subunit alpha; Neuroblastoma specific potassium channel subunit alpha KvLQT2; Neuroblastoma-specific potassium channel subunit alpha KvLQT2; Potassium voltage gated channel KQT like protein 2; Potassium voltage gated channel KQT like subfamily member 2; Potassium voltage gated channel subfamily KQT member 2; Potassium voltage-gated channel subfamily KQT member 2; Voltage gated potassium channel subunit Kv7.2; Voltage-gated potassium channel subunit Kv7.2.   
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 神經生物學  通道蛋白  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, 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 96kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KCNQ2 (91-150aa)
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:
Epilepsy affects about 0.5% of the world’s population and has a large genetic component. Epilepsy results from an electrical hyperexcitability in the central nervous system. Potassium channels are important regulators of electrical signaling, determining the firing properties and responsiveness of a variety of neurons. Benign familial neonatal convulsions (BFNC), an autosomal dominant epilepsy of infancy, has been shown to be caused by mutations in the KCNQ2 or the KCNQ3 potassium channel genes. KCNQ2 and KCNQ3 are voltage-gated potassium channel proteins with six putative transmembrane domains. Both proteins display a broad distribution within the brain, with expression patterns that largely overlap.

Function:
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.

Subunit:
Heteromultimer with KCNQ3. May associate with KCNE2.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Tissue Specificity:
In adult and fetal brain. Highly expressed in areas containing neuronal cell bodies, low in spinal chord and corpus callosum. Isoform 2 is preferentially expressed in differentiated neurons. Isoform 6 is prominent in fetal brain, undifferentiated neuroblastoma cells and brain tumors.

Post-translational modifications:
In Xenopus oocytes KCNQ2/KCNQ3 heteromeric current can be increased by intracellular cyclic AMP, an effect that depends on phosphorylation of Ser-52 in the N-terminus region.

DISEASE:
Defects in KCNQ2 are the cause of benign familial neonatal seizures type 1 (BFNS1) [MIM:121200]. 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) [MIM:613720]. EIEE7 is an autosomal dominant seizure disorder characterized by infantile onset of refractory seizures with resultant delayed neurologic development and persistent neurologic abnormalities.

Similarity:
Belongs to the potassium channel family. KQT (TC 1.A.1.15) subfamily. Kv7.2/KCNQ2 sub-subfamily.

Database links:

Entrez Gene: 3785 Human

Entrez Gene: 16536 Mouse

Entrez Gene: 170848 Rat

Omim: 602235 Human

SwissProt: O43526 Human

SwissProt: Q9Z351 Mouse

SwissProt: O88943 Rat

Unigene: 161851 Human

Unigene: 40615 Mouse

Unigene: 440175 Mouse

Unigene: 33317 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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