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Rabbit Anti-CHRNB/PE Conjugated antibody (bs-19241R-PE)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-19241R-PE
英文名稱 Rabbit Anti-CHRNB/PE Conjugated antibody
中文名稱 PE標記的煙堿型乙酰膽堿受體β1抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 免疫學  神經生物學  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHRNB
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.
產品介紹 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.
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