Overview

  • Product name

    Anti-CHRND antibody - C-terminal
    See all CHRND primary antibodies
  • Description

    Rabbit polyclonal to CHRND - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide, corresponding to a region within C terminal amino acids 453-517 of Human CHRND (Q07001).

  • Positive control

    • HepG2 cell lysate; A431 cells; Molt-4, 293T, H1299, and HeLaS3 cell lines.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • Storage buffer

    pH: 7.00
    Preservative: 0.01% Thimerosal (merthiolate)
    Constituents: 1.21% Tris, 0.75% Glycine, 20% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab137536 in the following tested applications.

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

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 59 kDa.
ICC/IF 1/100 - 1/1000.

Target

  • 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.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Delta/CHRND sub-subfamily.
  • Cellular localization

    Cell junction > synapse > postsynaptic cell membrane. Cell membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Anti-CHRND antibody - C-terminal (ab137536) at 1/1000 dilution + HepG2 cell lysate at 30 µg

    Predicted band size: 59 kDa

  • Immunofluorescence analysis of methanol-fixed A431, labelling CHRND with ab137536 at 1/200 dilution.

References

ab137536 has not yet been referenced specifically in any publications.

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