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货号： bs-19241R-PE-Cy5.5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19241R-PE-Cy5.5
- 英文名称
- Anti-CHRNB/PE-Cy5.5
- 中文名称
- PE-Cy5.5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: 282179Cow
Entrez Gene: 1140Human
Entrez Gene: 11443Mouse
Entrez Gene: 24261Rat
GenBank: NP_000738.2Human
Omim: 100710Human
SwissProt: P04758Cow
SwissProt: P11230Human
SwissProt: P09690Mouse
SwissProt: P25109Rat
Unigene: 330386Human
Unigene: 86425Mouse
Unigene: 44611Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.