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Rabbit Anti-SCN4B  antibody (bs-20042R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-20042R
英文名稱 Rabbit Anti-SCN4B  antibody
中文名稱 鈉通道亞基β4抗體
別    名 SCN4B_HUMAN. Sodium channel subunit beta-4.  
研究領域 心血管  神經生物學  信號轉導  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 22kDa
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCN4B: 21-100/228 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 The SCN4B protein modulates channel gating kinetics. Causes negative shifts in the voltage dependence of activation of certain alpha sodium channels, but does not affect the voltage dependence of inactivation By similarity. The protein has been found to be expressed at a high level in dorsal root ganglia, at a lower level in brain, spinal cord, skeletal muscle and heart. Defects in SCN4B are the cause of long QT syndrome type 10 (LQT10) [MIM:611819]. 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 excercise or emotional stress. They can present with a sentinel event of sudden cardiac death in infancy.

Function:
Modulates channel gating kinetics. Causes negative shifts in the voltage dependence of activation of certain alpha sodium channels, but does not affect the voltage dependence of inactivation.

Subunit:
The voltage-sensitive sodium channel consists of an ion conducting pore forming alpha-subunit regulated by one or more beta-1, beta-2, beta-3 and/or beta-4 subunits. Beta-1 and beta-3 are non-covalently associated with alpha, while beta-2 and beta-4 are covalently linked by disulfide bonds. Associates with SCN2A.

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

Tissue Specificity:
Expressed at a high level in dorsal root ganglia, at a lower level in brain, spinal cord, skeletal muscle and heart.

Post-translational modifications:
Contains a number of interchain disulfide bonds with SCN2A.

DISEASE:
Defects in SCN4B are the cause of long QT syndrome type 10 (LQT10) [MIM:611819]. 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 excercise or emotional stress. They can present with a sentinel event of sudden cardiac death in infancy.

Similarity:
Belongs to the sodium channel auxiliary subunit SCN4B (TC 8.A.17) family.
Contains 1 Ig-like C2-type (immunoglobulin-like) domain.

SWISS:
Q8IWT1

Gene ID:
6330

Database links:

Entrez Gene: 6330 Human

SwissProt: Q8IWT1 Human



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