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anti-AChR gamma antibody :: Mouse AChR gamma Monoclonal Antibody

Scan QR to view Datasheet Catalog #    MBS531011 anti-AChR gamma antibody
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 Product Name   

AChR gamma, Monoclonal Antibody

★Popular Item★
 Also Known As   

AChR gamma antibody

 Product Synonym Names    Monoclonal AChR gamma; Anti-AChR gamma; Acetylcholine Receptor gamma
 Product Gene Name   

anti-AChR gamma antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    100725
 3D Structure    ModBase 3D Structure for Q04844
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 Clonality    Monoclonal
 Isotype    IgG1
 Clone Number    223630
 Host    Mouse
 Species Reactivity    Specific for Human Acetylcholine Receptor. Does not cross react with Acetylcholine Receptor from mouse, rat or chick adult.
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 Form/Format    Ascites, supplied as a liquid in PBS, pH 7.4, with 0.1% NaN3
 Concentration    100ul (lot specific)
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 Biological Significance    An acetylcholine receptor (abbreviated AChR) is an integral membrane protein that responds to the binding of acetylcholine, a neurotransmitter. Like other transmembrane receptors, acetylcholine receptors are classified according to their "pharmacology", or according to their relative affinities and sensitivities to different molecules. Nicotinic acetylcholine receptors (nAChR), are particularly responsive to nicotine. Muscarinic acetylcholine receptors (mAChR), are particularly responsive to muscarine.
 Immunogen    Human Acetycholine Receptor
 Biohazard Information    This product contains sodium azide as preservative. Although the amount of sodium azide is very small appropriate care must be taken when handling.
    
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 Preparation and Storage    Store 4 degree C short term. Store at -20 degree C long term. Avoid repeated Freeze/Thaw cycles
 Other Notes    Small volumes of anti-AChR gamma antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for anti-AChR gamma antibody

   Mouse monoclonal AChR gamma antibody
 Product Categories/Family for anti-AChR gamma antibody    Neuroscience
 Applications Tested/Suitable for anti-AChR gamma antibody   

IHC; IP; WB

 Application Notes for anti-AChR gamma antibody    IHC: 1:3
IP: 1:100
WB: 1:100
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NCBI/Uniprot data below describe general gene information for AChR gamma. It may not necessarily be applicable to this product.
 NCBI GI #    4557463
 NCBI GeneID    1145
 NCBI Accession #    NP_000071.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000080.3 [Other Products]
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 UniProt Primary Accession #    Q04844 [Other Products]
 UniProt Secondary Accession #    D3DTK6 [Other Products]
 UniProt Related Accession #    Q04844 [Other Products]
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 NCBI Official Full Name    acetylcholine receptor subunit epsilon
 NCBI Official Synonym Full Names    cholinergic receptor, nicotinic, epsilon (muscle)
 NCBI Official Symbol    CHRNE [Similar Products]
 NCBI Official Synonym Symbols   
ACHRE; CMS1D; CMS1E; CMS2A; CMS4A; CMS4B; CMS4C; FCCMS; SCCMS
[Similar Products]
 NCBI Protein Information    acetylcholine receptor subunit epsilon
 UniProt Protein Name    Acetylcholine receptor subunit epsilon
 UniProt Gene Name    CHRNE [Similar Products]
 UniProt Synonym Gene Names    ACHRE [Similar Products]
 UniProt Entry Name    ACHE_HUMAN
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 NCBI Summary for AChR gamma    Acetylcholine receptors at mature mammalian neuromuscular junctions are pentameric protein complexes composed of four subunits in the ratio of two alpha subunits to one beta, one epsilon, and one delta subunit. The acetylcholine receptor changes subunit composition shortly after birth when the epsilon subunit replaces the gamma subunit seen in embryonic receptors. Mutations in the epsilon subunit are associated with congenital myasthenic syndrome. [provided by RefSeq, Sep 2009]
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 UniProt Comments for AChR gamma    nAChRE: 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. The muscle AChR is the major target antigen in the autoimmune disease 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 CHRNE are a cause of congenital myasthenic syndrome slow-channel type (SCCMS). 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 CHRNE are a cause of congenital myasthenic syndrome fast-channel type (FCCMS). 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. Defects in CHRNE are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (CMS-ACHRD). CMS-ACHRD is a postsynaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Epsilon/CHRNE sub-subfamily.

Protein type: Membrane protein, integral; Channel, ligand-gated; Channel, cation; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 17p13.2

Cellular Component: nicotinic acetylcholine-gated receptor-channel complex; postsynaptic membrane; integral to plasma membrane; plasma membrane; cell junction

Molecular Function: cation transmembrane transporter activity; acetylcholine receptor activity; nicotinic acetylcholine-activated cation-selective channel activity

Biological Process: synaptic transmission; regulation of membrane potential; muscle contraction; transport; signal transduction; synaptic transmission, cholinergic

Disease: Myasthenic Syndrome, Congenital, 4b, Fast-channel; Myasthenic Syndrome, Congenital, Associated With Acetylcholine Receptor Deficiency; Myasthenic Syndrome, Congenital, 4a, Slow-channel
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 Research Articles on AChR gamma    1. mutational analysis of CHRNE revealed a homozygous 1293insG, which is a well-known low-expressor receptor mutation in patients with epidermolysis bullosa simplex and congenital myasthenic syndrome.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-AChR gamma antibodyPathways associated with anti-AChR gamma antibody
 Reference Product  PubMed Publications
 CHRNA1 antibody  >5 publications with AChR gamma and CHRNA1
 CHRNB1 antibody  >3 publications with AChR gamma and CHRNB1
 JUN antibody  >3 publications with AChR gamma and JUN
 ACHE antibody  >2 publications with AChR gamma and ACHE
 Products by Pathway  Pathway Diagram
 Acetylcholine Binding And Downstream Events Pathway antibodies  Acetylcholine Binding And Downstream Events Pathway Diagram
 Activation Of Nicotinic Acetylcholine Receptors Pathway antibodies  Activation Of Nicotinic Acetylcholine Receptors Pathway Diagram
 ErbB2/ErbB3 Signaling Events Pathway antibodies  ErbB2/ErbB3 Signaling Events Pathway Diagram
 Highly Sodium Permeable Acetylcholine Nicotinic Receptors Pathway antibodies  Highly Sodium Permeable Acetylcholine Nicotinic Receptors Pathway Diagram
 Neuroactive Ligand-receptor Interaction Pathway antibodies  Neuroactive Ligand-receptor Interaction Pathway Diagram
 Neuroactive Ligand-receptor Interaction Pathway antibodies  Neuroactive Ligand-receptor Interaction Pathway Diagram
 Neuronal System Pathway antibodies  Neuronal System Pathway Diagram
 Neurotransmitter Receptor Binding And Downstream Transmission In The Postsynaptic Cell Pathway antibodies  Neurotransmitter Receptor Binding And Downstream Transmission In The Postsynaptic Cell Pathway Diagram
 Postsynaptic Nicotinic Acetylcholine Receptors Pathway antibodies  Postsynaptic Nicotinic Acetylcholine Receptors Pathway Diagram
 Presynaptic Nicotinic Acetylcholine Receptors Pathway antibodies  Presynaptic Nicotinic Acetylcholine Receptors Pathway Diagram
Diseases associated with anti-AChR gamma antibodyOrgans/Tissues associated with anti-AChR gamma antibody
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >35 publications with AChR gamma and Nervous System Diseases
 Autoimmune Diseases Antibodies  >5 publications with AChR gamma and Autoimmune Diseases
 Myasthenic Syndrome, Congenital, Fast-Channel Antibodies  >4 publications with AChR gamma and Myasthenic Syndrome, Congenital, Fast-Channel
 Pregnancy Complications Antibodies  >1 publications with AChR gamma and Pregnancy Complications
 Myasthenic syndrome, congenital, postsynaptic slow-channel Antibodies  >1 publications with AChR gamma and Myasthenic syndrome, congenital, postsynaptic slow-channel
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >4 publications with AChR gamma and Brain
 Eye Antibodies  >1 publications with AChR gamma and Eye
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