Anti-CHRNE/FITC Conjugated抗体上海钰博生物科技有限公司

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产品名称: Anti-CHRNE/FITC Conjugated抗体
英文名称: Anti-CHRNE/FITC
抗体货号: YB--19244R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human CHRNE
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/7/11 11:50:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-CHRNE/FITC Conjugated抗体

品编号 YB-19244R-FITC
英文名称 Anti-CHRNE/FITC
中文名称 FITC标记的烟碱型乙酰胆碱受体ε抗体
别    名 Nicotinic Acetylcholine Receptor epsilon; Acetylcholine receptor subunit epsilon; ACHE_HUMAN; AchR epsilon subunit; ACHRE; Cholinergic receptor, nicotinic, epsilon polypeptide; Chrne; CMS1D; CMS1E; CMS2A; FCCMS; SCCMS.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  细胞膜受体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 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 53kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHRNE
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 background:
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. [provided by RefSeq, Sep 2009]

Function:
After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.

Subunit:
Pentamer of two alpha chains, and one each of the beta, delta, and gamma (in immature muscle) or epsilon (in mature muscle) chains.

Subcellular Location:
Cell junction; synapse; postsynaptic cell membrane. Cell membrane.

DISEASE:
Note=The muscle AChR is the major target antigen in the autoimmune disease myasthenia gravis. Myasthenia gravis is characterized by sporadic muscular fatigability and weakness, occurring chiefly in muscles innervated by cranial nerves, and characteristically improved by cholinesterase-inhibiting drugs. Defects in CHRNE are a cause of congenital myasthenic syndrome slow-channel type (SCCMS) [MIM:601462]. SCCMS is the most common congenital myasthenic syndrome. Congenital myasthenic syndromes are characterized by muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. SCCMS is caused by kinetic abnormalities of the AChR, resulting in prolonged endplate currents and prolonged AChR channel opening episodes.
Defects in CHRNE are a cause of congenital myasthenic syndrome fast-channel type (FCCMS) [MIM:608930]. FCCMS is a congenital myasthenic syndrome characterized by kinetic abnormalities of the AChR. In most cases, FCCMS is due to mutations that decrease activity of the AChR by slowing the rate of opening of the receptor channel, speeding the rate of closure of the channel, or decreasing the number of openings of the channel during ACh occupancy. The result is failure to achieve threshold depolarization of the endplate and consequent failure to fire an action potential.
Defects in CHRNE are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (CMS-ACHRD) [MIM:608931]. CMS-ACHRD is a postsynaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance.

Similarity:
Belongs to the ligand-gated ion channel (TC 1.A.9) family. 
Acetylcholine receptor (TC 1.A.9.1) subfamily.
Epsilon/CHRNE sub-subfamily.

Database links:

Entrez Gene: 1145 Human

Entrez Gene: 281688 Cow

Entrez Gene: 101137825 Gorilla

Entrez Gene: 100146223 Horse

Entrez Gene: 11448 Mouse

Entrez Gene: 29422 Rat

Entrez Gene: 710301 Rhesus monkey

GenBank: NP_033733.1 Mouse

Omim: 100725 Human

SwissProt: P02715 Cow

SwissProt: Q04844 Human

SwissProt: P20782 Mouse

SwissProt: P09660 Rat

Unigene: 654535 Human

Unigene: 4980 Mouse

Unigene: 10301 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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