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merck millipore,默克密理博,AB5936,Anti-Acetylcholine Receptor-γ Antibody
产品名称：Anti-Acetylcholine Receptor-γ Antibody
产品型号：AB5936
This Anti-Acetylcholine Receptor-γ Antibody is validated for use in IH for the detection of Acetylcholine Receptor-γ.
merck millipore,默克密理博,AB5936,Anti-Acetylcholine Receptor-γ 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 M IHC Ch Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB5936 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Acetylcholine Receptor-γ Antibody 产品信息 格式 Affinity Purified 演示 Affinity purified chicken IgY. Liquid. 应用 应用 This Anti-Acetylcholine Receptor-γ Antibody is validated for use in IH for the detection of Acetylcholine Receptor-γ. 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 gamma. 品种反应性 Mouse 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_005199.4
Entrez基因汇总 For background information on the acetylcholine receptor (AChR), see CHRNA1 (MIM 100690). Two forms of AChR are found in mammalian skeletal muscle cells. The mature form is predominant in innervated adult muscle and the embryonic form is present in fetal and denervated muscle. Embryonic and mature AChR differ by the replacement of the gamma subunit in the pentameric glycoprotein complex by its isoform, the epsilon subunit (MIM 100725), which is specific to the mature AChR subtype. This switch is mediated by ARIA (acetylcholine receptor-inducing activity; MIM 142445).[supplied by OMIM] 基因符号 - ACHRG
- CHRNG
- MGC133376
UniProt编号 - P07510
UniProt汇总 FUNCTION: SwissProt: P07510 # 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: 517 amino acids; 57883 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: P07510 # Defects in CHRNG 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. Inheritance can be autosomal dominant, autosomal recessive, or X linked, but autosomal recessive inheritance appears to be most common. Clinical expression is very variable, and, in the severest form, lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia (e.g., chin to sternum, cervical, axillary, humero-ulnar, crural, popliteal, and ankles), 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-in particular, cleft palate-are frequent. & Defects in CHRNG are a cause of Escobar syndrome [MIM:265000]; also called Escobar variant multiple pterygium syndrome or nonlethal type multiple pterygium syndrome. Escobar syndrome is a nonlethal form of arthrogryposis multiplex congenita. It is an autosomal recessive condition characterized by excessive webbing (pterygia), congenital contractures (arthrogryposis), and scoliosis. Variable other features include intrauterine death, congenital respiratory distress, short stature, faciocranial dysmorphism, ptosis, low-set ears, arachnodactyly and cryptorchism in males. Congenital contractures are common and may be caused by reduced fetal movements at sensitive times of development. Possible causes of decreased fetal mobility include space constraints such as oligohydramnion, drugs, metabolic conditions or neuromuscular disorders including myasthenia gravis.
SIMILARITY: SwissProt: P07510 ## 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 -20°C in undiluted aliquots for up to 6 months. Avoid repeated freeze/thaw cycles. Glycerol (1:1, ACS or better grade) can be added for additional stability. 包装信息 数量 100 µg
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