- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
- 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 M IHC Ch Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB5938 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Acetylcholine Receptor-ε Antibody 产品信息 格式 Affinity Purified 演示 Affinity purified chicken IgY. Liquid. 应用 应用 Detect Acetylcholine Receptor-ε using this Anti-Acetylcholine Receptor-ε Antibody validated for use in IH. 主要应用 - Immunohistochemistry
应用说明 Optimal working dilutions must be determined by end user. 生物信息 免疫原品种 Synthetic peptide. 宿主 Chicken 特异性 Acetylcholine Receptor epsilon. 品种反应性 Mouse 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_000080.2
Entrez基因汇总 Acetylcholine receptors at mature mammalian neuromuscular junctions are pentameric protein complexes composed of four subunits in the ratio of two alpha subunits to one beta, one epsilon, and one delta subunit. The achetylcholine receptor changes subunit composition shortly after birth when the epsilon subunit replaces the gamma subunit seen in embryonic receptors. Mutations in the epsilon subunit are associated with congenital myasthenic syndrome. 基因符号 - SCCMS
- CHRNE
- FCCMS
- ACHRE
- CMS1E
- CMS1D
- CMS2A
UniProt编号 - Q04844
UniProt汇总 FUNCTION: SwissProt: Q04844 # 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.
SIZE: 493 amino acids; 54697 Da
SUBUNIT: 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.
DISEASE: SwissProt: Q04844 # The muscle AChR is the major target antigen in the autoimmune disease 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 CHRNE are a cause of slow-channel congenital myasthenic syndrome (SCCMS) [MIM:601462, 254200]. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie slow-channel myasthenic syndromes (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in fast-channel syndromes (FCCMS). A third disorder associated with postsynaptic CMS is called CMS type Id (CMS1d), and could also result from mutations in the proteins forming the subunits of the muscle AChR. Mutations underlying SCCMS cause a gain of function and usually show dominant inheritance. & Defects in CHRNE are a cause of fast-channel congenital myasthenic syndrome (FCCMS) [MIM:608930]. Mutations underlying FCCMS cause a loss of function and show recessive inheritance. & Defects in CHRNE are a cause of congenital myasthenic syndrome type Id (CMS1d) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. Mutations underlying AChR deficiency cause a loss of function and show recessive inheritance.
SIMILARITY: SwissProt: Q04844 ## Belongs to the ligand-gated ionic channel (TC 1.A.9) family.产品使用声明 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 at 2-8°C in undiluted aliquots for up to 6 months after date of receipt. 包装信息 数量 100 µg
上一件merck millipore产品：merck millipore,默克密理博,492018,NP-40 Alternative, PROTEIN GRADE Detergent, 10% Solution, Sterile-Filtered - CAS 9016-45-9 - Calbiochem
下一件merck millipore产品：merck millipore,默克密理博,05-164,Anti-PLCβ-1 Antibody
相关产品
merck millipore,默克密理博,105360,L-亮胺酸
merck millipore,默克密理博,ABE499,Anti-JMJD2D Antibody
merck millipore,默克密理博,06-1096,Anti-IPS-1 Antibody
merck millipore,默克密理博,PSHT004S5,Millicell-96 插入式细胞培养皿，聚碳酸酯，0.4 µm
merck millipore,默克密理博,05-164,Anti-PLCβ-1 Antibody
merck millipore,默克密理博,05-945,Anti-NKG2D Antibody, clone 3.1.1.1
merck millipore,默克密理博,ABC347,Anti-Fn14 Antibody
merck millipore,默克密理博,IHCR1005-6,Anti-Tyrosine Hydroxylase Antibody, prediluted, clone LNC1
merck millipore,默克密理博,SCR006,Accumax cell detachment solution