Anti-CHRNE antibody [5F11G8]

Anti-CHRNE antibody [5F11G8] 

• 概述

• 产品描述Acetylcholine receptor subunit epsilon is a protein that in humans is encoded by the CHRNE gene. Acetylcholine receptors at mature mammalian neuromuscular junctions are pentameric protein complexes composed of four subunits in the ratio of two alpha subunits to one beta, one epsilon, and one delta subunit. The achetylcholine receptor changes subunit composition shortly after birth when the epsilon subunit replaces the gamma subunit seen in embryonic receptors. Mutations in the epsilon subunit are associated with congenital myasthenic syndrome. Congenital myasthenic syndrome (CMS) is associated with genetic defects that affect proteins of the neuromuscular junction. Postsynaptic defects are the most frequent cause of CMS and often result in abnormalities in the acetylcholine receptor (AChR). The majority of mutations causing CMS are found in the AChR subunits genes. Out of all mutations associated with CMS, more than half are mutations in one of the four genes encoding the adult AChR subunits. Mutations of the AChR often result in endplate deficiency. The most common AChR gene mutation that underlies CMS is the mutation of the CHRNE gene. The CHRNE gene codes for the epsilon subunit of the AChR. Most mutations are autosomal recessive loss-of-function mutations and as a result there is endplate AChR deficiency. CHRNE is associated with changing the kinetic properties of the AChR.
• 产品名称Anti-CHRNE antibody [5F11G8]
• 分子量54.7kDa
• 种属反应性Human,Rat
• 验证应用WB,FC
• 抗体类型小鼠单抗
• 免疫原Purified recombinant fragment of human CHRNE (AA: extra 21-239) expressed in E. Coli.
• 偶联Non-conjugated

• 性能

• 形态Liquid
• 浓度1 mg/mL
• 存放说明Store at +4℃ after thawing. Aliquot store at -20℃. Avoid repeated freeze / thaw cycles.
• 存储缓冲液1*PBS with 0.05% sodium azide.
• 亚型IgG1
• 纯化方式Protein G purified.
• 亚细胞定位Cell junction, synapse, postsynaptic cell membrane. Cell membrane.
• 其它名称
  • Acetylcholine receptor subunit epsilon antibody
  • ACHE_HUMAN antibody
  • AChR antibody
  more

• 应用

  WB: 1:500-1:2,000
  FC: 1:100-1:200

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  Fig1: Western blot analysis of CHRNE against human CHRNE (AA: extra 21-239) recombinant protein. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.

   

  

  Fig2: Western blot analysis of  against HEK293 (1) and CHRNE (AA: extra 21-239)-hIgGFc transfected HEK293 (2) cell lysate.Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody  was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.

   

  

  Fig3: Western blot analysis of  against C6 (1) cell lysate.Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.

   

  

  Fig4: Flow cytometric analysis of CHRNE was done on SK-N-SH cells. The cells were fixed, permeabilized and stained with the primary antibody (green). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated goat anti-Mouse IgG Secondary antibody at 1/500 dilution for 30 minutes. Unlabelled sample was used as a control (cells without incubation with primary antibody; red).

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