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merck millipore,默克密理博,AB15324,Anti-Nicotinic Acetylcholine Receptor α6 Antibody
产品名称:Anti-Nicotinic Acetylcholine Receptor α6 Antibody
产品型号:AB15324
Anti-Nicotinic Acetylcholine Receptor α6 Antibody is an antibody against Nicotinic Acetylcholine Receptor α6 for use in WB.
merck millipore,默克密理博,AB15324,Anti-Nicotinic Acetylcholine Receptor α6 Antibody
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H, M, R WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB15324 描述 Anti-Nicotinic Acetylcholine Receptor α6 Antibody Alternate Names - nicotinic cholinergic receptor alpha 1
- cholinergic receptor, nicotinic, alpha 1 (muscle)
- cholinergic receptor, nicotinic, alpha polypeptide 1
- (muscle)
- nicotinic acetylcholine receptor alpha subunit
背景信息 Nicotinic acetylcholine receptors, or nAChRs, are cholinergic receptors that form ligand-gated ion channels in the plasma membranes of certain neurons. nAChR is triggered by the binding of the neurotransmitter acetylcholine (ACh) and is also opened by nicotine. After binding ACh, the nAChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. nAChRs are present in many tissues in the body: the neuronal receptors are present in the central nervous system and the peripheral nervous system; and the neuromuscular receptors are found in the neuromuscular junctions of somatic muscles. Neuronal AChR is composed of two different types of subunits: alpha (α) and beta (β). 产品信息 格式 Affinity Purified 控制 - Rat e16 brain lysate
演示 Rabbit purified polyclonal serum in buffer containing 0.1 M Tris-Glycine (pH7.4) 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-Nicotinic Acetylcholine Receptor α6 Antibody is an antibody against Nicotinic Acetylcholine Receptor α6 for use in WB. 主要应用 - Western Blotting
生物信息 免疫原品种 This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from residues of Nicotinic Acetylcholine Receptor α6. 表位 Extracellular Domain 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes Nicotinic Acetylcholine Receptor α6. 品种反应性 HumanMouseRat Species Reactivity Note Demonstrated to react with human, mouse, and rat. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_476532
Entrez基因汇总 May form a nicotinic acetylcholine receptor; may play a role in fast synaptic transmission [RGD]. 基因符号 - SCCMS
- ACHRA
- ACHRD
- CHNRA
- CHRNA
- CMS2A
- FCCMS
修改 - Acetylation
纯化方法 Antigen affinity purified UniProt编号 - P02708
UniProt汇总 "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 STRUCTURE: 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; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.
TISSUE SPECIFICITY: Isoform 1 is only expressed in skeletal muscle whereas isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.
INVOLVEMENT IN DISEASE: Defects in CHRNA1 are a cause of lethal type multiple pterygium syndrome [MIM:253290]. Multiple pterygia are found infrequently in children with arthrogryposis and in fetuses with fetal akinesia syndrome. In lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia, and flexion contractures causing severe arthrogryposis and fetal akinesia. Subcutaneous edema can be severe, causing fetal hydrops with cystic hygroma and lung hypoplasia. Oligohydramnios and facial anomalies are frequent.
The alpha subunit is the main focus for antibody binding in myasthenia gravis [MIM:254200]. 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 CHRNA1 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 CHRNA1 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.
SEQUENCE SIMILARITIES: Belongs to the ligand-gated ionic channel (TC 1.A.9) family. [View classification]产品使用声明 质量保证 Evaluated by Western Blot in Rat e16 brain lysate.
Western Blot Analysis: : 1:10,000 dilution of this lot detected Nicotinic Acetylcholine Receptor α6 in 10 µg of e16 rat brain lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain refrigerated at 2-8°C for up to 12 months from date of receipt. 包装信息 数量 100 µg
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