Overview

  • Product name

    Anti-Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 antibody
    See all Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 primary antibodies
  • Description

    Rabbit polyclonal to Nicotinic Acetylcholine Receptor alpha 1/CHRNA1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Cat
  • Immunogen

    Recombinant fragment corresponding to Human Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 aa 189-250.
    Sequence:

    MKLGTWTYDGSVVAINPESDQPDLSNFMESGEWVIKESRGWKHSVTYSCC PDTPYLDITYHF


    Database link: P02708

  • Positive control

    • ICC/IF: RH-30 cells.
  • General notes

     This product was previously labelled as Nicotinic Acetylcholine Receptor alpha 1

     

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: 40% Glycerol, PBS
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab221868 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
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

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.
  • Tissue specificity

    Isoform 1 is only expressed in skeletal muscle. Isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.
  • Involvement in disease

    Defects in CHRNA1 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.
    Note=The alpha subunit is the main focus for antibody binding in myasthenia gravis. 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 CHRNA1 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 CHRNA1 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. Alpha-1/CHRNA1 sub-subfamily.
  • Cellular localization

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

  • Alternative names

    • Acetylcholine receptor subunit alpha antibody
    • ACHA_HUMAN antibody
    • AChR antibody
    • ACHRA antibody
    • ACHRD antibody
    • CHNRA antibody
    • Cholinergic receptor nicotinic alpha 1 subunit antibody
    • Cholinergic receptor nicotinic alpha polypeptide 1 antibody
    • Cholinergic receptor, nicotinic, alpha polypeptide 1 (muscle) antibody
    • Chrna1 antibody
    • CMS1A antibody
    • CMS1B antibody
    • CMS2A antibody
    • FCCMS antibody
    • Nicotinic cholinergic receptor alpha 1 antibody
    • SCCMS antibody
    • Schizophrenia neurophysiologic defect candidate antibody
    see all

Images

  • PFA-fixed, Triton X-100 permeabilized RH-30 cells stained for Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 (green) using ab221868 at 4 µg/ml in ICC/IF.

References

ab221868 has not yet been referenced specifically in any publications.

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