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Rabbit Anti-KCNE1/AP Conjugated antibody (bs-9928R-AP)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-9928R-AP
英文名稱(chēng)1 Rabbit Anti-KCNE1/AP Conjugated antibody
中文名稱(chēng) 堿性磷酸酶(AP)標(biāo)記的鉀離子通道蛋白家族成員1抗體
別    名 Delayed rectifier potassium channel subunit IsK; Human cardiac delayed rectifier potassium channel protein; IKs producing slow voltage gated potassium channel subunit beta Mink; ISK; JLNS 2; JLNS; JLNS2; KCNE 1; LQT 5; LQT5; Minimal potassium channel; MinK; Potassium voltage gated channel Isk related family member 1; Potassium voltage gated channel Isk related subfamily member 1; Potassium voltage gated channel subfamily E member 1; KCNE1_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 心血管  神經(jīng)生物學(xué)  通道蛋白  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 15kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KCNE1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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(chǎn)品介紹 background:
The product of this gene belongs to the potassium channel KCNE family. Potassium ion channels are essential to many cellular functions and show a high degree of diversity, varying in their electrophysiologic and pharmacologic properties. This gene encodes a transmembrane protein known to associate with the product of the KVLQT1 gene to form the delayed rectifier potassium channel. Mutation in this gene are associated with both Jervell and Lange-Nielsen and Romano-Ward forms of long-QT syndrome. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008].

Function:
Ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. Modulates the gating kinetics and enhances stability of the channel complex. Assembled with KCNQ1/KVLQT1 is proposed to form the slowly activating delayed rectifier cardiac potassium (IKs) channel. The outward current reaches its steady state only after 50 seconds. Assembled with KCNH2/HERG may modulate the rapidly activating component of the delayed rectifying potassium current in heart (IKr).

Subunit:
Associates with KCNQ1/KVLQT1 and KCNH2/HERG.

Subcellular Location:
Membrane; Single-pass type I membrane protein.

Tissue Specificity:
Expressed in heart, lung, kidney, testis, ovaries, small intestine, peripheral blood leukocytes. Not detected in pancreas, spleen, prostate and colon. Restrictively localized in the apical membrane portion of epithelial cells.

Post-translational modifications:
Phosphorylation inhibits the potassium current (By similarity).
N-glycosylation at Asn-26 occurs post-translationally, and requires prior cotranslational glycosylation at Asn-5.

DISEASE:
Defects in KCNE1 are the cause of Jervell and Lange-Nielsen syndrome type 2 (JLNS2) [MIM:612347]. JLNS2 is an autosomal recessive disorder characterized by congenital deafness, prolongation of the QT interval, syncopal attacks due to ventricular arrhythmias, and a high risk of sudden death.
Defects in KCNE1 are the cause of long QT syndrome type 5 (LQT5) [MIM:613695]. Long QT syndromes are heart disorders characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress. KCNE1 mutants form channels that open slowly and close rapidly, thereby diminishing potassium currents.

Similarity:
Belongs to the potassium channel KCNE family.

Database links:

Entrez Gene: 3753 Human

Entrez Gene: 16509 Mouse

Entrez Gene: 397331 Pig

Entrez Gene: 25471 Rat

Omim: 176261 Human

SwissProt: P15382 Human

SwissProt: P23299 Mouse

SwissProt: Q9TUH9 Pig

SwissProt: P15383 Rat

Unigene: 121495 Human

Unigene: 299425 Mouse

Unigene: 9734 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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