概述

  • 产品名称Anti-CHRND抗体
    参阅全部 CHRND 一抗
  • 描述
    兔多克隆抗体to CHRND
  • 经测试应用适用于: ELISA, WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Rat, Cow, Dog
  • 免疫原

    Synthetic peptide derived from a region between residues 6-55 of human Acetylcholine Receptor Protein delta Precursor. (the amino acid sequence is considered to be commercially sensitive)

  • 阳性对照
    • Jurkat cell lysate (WB), Human Kidney (IHC)

性能

应用

Our Abpromise guarantee covers the use of ab26095 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
ELISA 1/62500.
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 59 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
IHC-P Use at an assay dependent concentration.

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 CHRND 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 CHRND 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 CHRND 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.
  • 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. Delta/CHRND 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 delta subunit antibody
    • Acetylcholine receptor subunit delta antibody
    • ACHD_HUMAN antibody
    • ACHRD antibody
    • Cholinergic receptor, nicotinic, delta polypeptide antibody
    • CHRND antibody
    • CMS2A antibody
    • FCCMS antibody
    • Nicotinic acetylcholine receptor delta polypeptide precursor antibody
    • SCCMS antibody
    see all

Anti-CHRND antibody 图像

  • Anti-CHRND antibody (ab26095) (at 1 ug/ml in 5% skim milk / PBS buffer) + Jurkat cell lysate 10ug.

    Secondary
    HRP conjugated anti Rabbit IgG diluted in 1/50,000 - 1/100,000.

    Predicted band size : 59 kDa
  • Ab26095 at a concentration of 4-8ug/ml staining paraffin embedded human kidney tissue by Immunohistochemistry. The arrows indicate the epithelial cells of renal tubule. Magnification: x400.

Anti-CHRND antibody (ab26095)参考文献

This product has been referenced in:
  • Soeda J  et al. Nicotine induces fibrogenic changes in human liver via nicotinic acetylcholine receptors expressed on hepatic stellate cells. Biochem Biophys Res Commun : (2011). Read more (PubMed: 22108052) »

See 1 Publication for this product

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