CHRNE Mouse Monoclonal Antibody [5F11G8]
Catalog# EM1711-01
CHRNE Mouse Monoclonal Antibody [5F11G8]
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WB
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FC
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Human
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Rat
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unconjugated
概述
产品名称
CHRNE Mouse Monoclonal Antibody [5F11G8]
抗体类型
Mouse Monoclonal Antibody
免疫原
Purified recombinant fragment of human CHRNE (AA: extra 21-239) expressed in E. Coli.
种属反应性
Human, Rat
验证应用
WB, FC
分子量
54.7kDa
阳性对照
C6 cell SK-N-SH cells 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 acetylcholine 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.
偶联
unconjugated
克隆号
5F11G8
产品特性
形态
Liquid
浓度
存放说明
4℃; -20℃ for long term storage.
存储缓冲液
Purified antibody in PBS with 0.05% sodium azide.
亚型
IgG1
纯化方式
Protein G affinity purified.
应用稀释度
-
WB
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1:500-1:2,000
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FC
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1:100-1:200
靶点
功能
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.
背景文献
1. J Neuroophthalmol. 2011 Mar;31(1):42-7.
2. Neurology. 2008 Dec 9;71(24):1967-72.
亚细胞定位
Cell junction, synapse, postsynaptic cell membrane. Cell membrane.
UNIPROT
别名
Acetylcholine receptor subunit epsilon antibody
ACHE_HUMAN antibody
AChR antibody
AchR epsilon subunit antibody
ACHRE antibody
Cholinergic receptor
nicotinic
epsilon polypeptide antibody
Chrne antibody
CMS1D antibody
展开图片
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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 (EM1711-01, 1/500) 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.
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Western blot analysis of EM1711-01 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 (EM1711-01, 1/500) 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.
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Western blot analysis of EM1711-01 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 (EM1711-01, 1/500) 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.
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Flow cytometric analysis of CHRNE was done on SK-N-SH cells. The cells were fixed, permeabilized and stained with the primary antibody (EM1711-01, 1/100) (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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