Anti-Nicotinic Acetylcholine Receptor gamma抗体[66] (ab24667)

概述

  • 产品名称Anti-Nicotinic Acetylcholine Receptor gamma抗体[66]
    参阅全部 Nicotinic Acetylcholine Receptor gamma 一抗
  • 描述
    大鼠单克隆抗体[66] to Nicotinic Acetylcholine Receptor gamma
  • 特异性ab24667 recognises Nicotinic Acetylcholine Receptor gamma .
  • 经测试应用适用于: WB, IP, ICC/IFmore details
  • 种属反应性
    与反应: Cow, Human
    不与反应: Rat
  • 免疫原

    Full length native protein (purified) (Cow): purified bovine muscle nicotinic acetylcholine receptor.

  • 常规说明


    ab24667 is useful to study specificities of autoantibodies in myasthenia gravis (MG) patient sera.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: None
    Constituents: Ascites
  • Concentration information loading...
  • 纯度Ascites
  • Primary antibody说明ab24667 is useful to study specificities of autoantibodies in myasthenia gravis (MG) patient sera.
  • 克隆单克隆
  • 克隆编号66
  • 同种型IgG2a
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab24667 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB 1/500 - 1/1000. Predicted molecular weight: 58 kDa.
IP 1/100.
ICC/IF 1/250 - 1/500.

靶标

  • 功能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.
  • 疾病相关Defects in CHRNG are a cause of multiple pterygium syndrome lethal type (MUPSL) [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.
    Defects in CHRNG are a cause of multiple pterygium syndrome Escobar variant (MUPSE) [MIM:265000]; also known as nonlethal type multiple pterygium syndrome. Escobar syndrome is a non-lethal 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. is a.
  • 序列相似性Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Gamma/CHRNG sub-subfamily.
  • 细胞定位Cell junction > synapse > postsynaptic cell membrane. Cell membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Acetylcholine receptor muscle gamma subunit antibody
    • Acetylcholine receptor protein gamma chain precursor antibody
    • Acetylcholine receptor subunit gamma antibody
    • ACHG antibody
    • ACHG_HUMAN antibody
    • Achr 3 antibody
    • AChR antibody
    • Achr3 antibody
    • ACHRG antibody
    • ACRG antibody
    • Cholinergic receptor nicotinic gamma antibody
    • Cholinergic receptor nicotinic gamma polypeptide antibody
    • CHRNG antibody
    • MGC133376 antibody
    see all

Anti-Nicotinic Acetylcholine Receptor gamma antibody [66] (ab24667)参考文献

ab24667 has not yet been referenced specifically in any publications.

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