烟碱型乙酰胆碱受体β1抗体
规格：1mg/1ml
英文名： CHRNB
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 (mu
分子量： 57kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CHRNB
交叉反应：Human, Mouse, Rat, Cow,
细胞定位：细胞膜 分泌型蛋白
烟碱型乙酰胆碱受体β1抗体产品介绍：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), 烟碱型乙酰胆碱受体β1抗体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) 烟碱型乙酰胆碱受体β1抗体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.
烟碱型乙酰胆碱受体β1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：**学 神经生物学 细胞膜受体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid