超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
91福利影视,国产91区精品福利在线社区,亚洲国产长腿丝袜AV天堂
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-CHRNE/PE Conjugated antibody (bs-19244R-PE)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-19244R-PE
英文名稱 Rabbit Anti-CHRNE/PE Conjugated antibody
中文名稱 PE標記的煙堿型乙酰膽堿受體ε抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細胞生物  神經(jīng)生物學(xué)  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHRNE
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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:
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.
版權(quán)所有 2004-2026 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
主站蜘蛛池模板: 最近中文字幕第一页 | 成人免费视频一区二区 | 风流少妇树林打野战视频 | 国产欧美久久久精品影院 | 男人操女人视频免费观看 | 91婷婷韩国欧美一区二区 | 人与禽交av在线播放 | 黄网站网址大全免费的 | 中文字幕制服丝袜第57页 | 99热最新网址获取 | 国产亚洲综合久久系列 | 亚洲欧美国产一区二区 | 男人av的天堂无码专区 | 精品一久久 | 99国产精品久久久久99打野战 | 欧美日韩a级片 | 无码毛片视频一区二区三区 | 国产欧美久久久精品影院 | 精品久久久久一区二区国产 | 爱情岛论坛网址永久首页 | 欧美精品高清在线观看 | 91操视频 | 草久影院 | 成人国产精品高清在线观看 | 精品自拍一区 | 日本动漫爆乳h动漫啪啪免费 | 久久精品爱爱视频 | av在线亚洲男人的天堂 | 色94色欧美sute亚洲线路二 | 日韩无砖专区一中文字目 | 国产精品高清视亚洲精品 | 亚洲综合激情五月丁香六月 | 国产成人精品一区二区免费看京 | 三级黄色理论片 | 欧美激情高清 | 日本成a人片在线观看日本 久久婷婷五月综合色国产免费观看 | 人人综合亚洲无线码另类 | 亚洲五月综合缴情在线观看 | 激情内射日本一区二区三区 | 国产呦精品一区二区三区网站 | 精品国产一区一区二区三亚瑟 |