Anti-CHRNB/FITC Conjugated抗体-抗体-抗体-生物在线
Anti-CHRNB/FITC Conjugated抗体

Anti-CHRNB/FITC Conjugated抗体

商家询价

产品名称: Anti-CHRNB/FITC Conjugated抗体

英文名称: Anti-CHRNB/FITC

产品编号: YB--19241R-FITC

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

上海钰博生物科技有限公司
  • 联系人 : 陈环环
  • 地址 : 上海市沪闵路6088号龙之梦大厦8楼806室
  • 邮编 : 200612
  • 所在区域 : 上海
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  • 邮箱 : shybio@126.com

 Anti-CHRNB/FITC Conjugated抗体

产品编号 YB-19241R-FITC
英文名称 Anti-CHRNB/FITC
中文名称 FITC标记的烟碱型乙酰胆碱受体β1抗体
别    名 Nicotinic Acetylcholine Receptor beta Acetylcholine receptor protein beta chain precursor; Acetylcholine receptor subunit beta; ACHB_HUMAN; ACHRB; Cholinergic receptor nicotinic beta polypeptide 1 (muscle); Chrnb 1; CHRNB; Chrnb1; CMS1D; CMS2A; Nicotinic acetylcholine receptor beta subunit precursor; SCCMS.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 免疫学  神经生物学  细胞膜受体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应  
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 55kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHRNB
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:
The muscle acetylcholine receptor is composed of five subunits: two alpha subunits and one beta, one gamma, and one delta subunit. This gene encodes the beta subunit of the acetylcholine receptor. The acetylcholine receptor changes conformation upon acetylcholine binding leading to the opening of an ion-conducting channel across the plasma membrane. Mutations in this gene are associated with slow-channel congenital myasthenic syndrome. [provided by RefSeq, Jul 2008]

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.

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

DISEASE:
Defects in CHRNB1 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 CHRNB1 are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (ACHRDCMS) [MIM:608931]. ACHRDCMS is a post-synaptic 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. Beta-1/CHRNB1 sub-subfamily.

Database links:

Entrez Gene: 282179 Cow

Entrez Gene: 1140 Human

Entrez Gene: 11443 Mouse

Entrez Gene: 24261 Rat

GenBank: NP_000738.2 Human

Omim: 100710 Human

SwissProt: P04758 Cow

SwissProt: P11230 Human

SwissProt: P09690 Mouse

SwissProt: P25109 Rat

Unigene: 330386 Human

Unigene: 86425 Mouse

Unigene: 44611 Rat



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