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

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Catalog # MBS531011
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anti-AChR gamma antibody
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
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
493
OMIM
100725
3D Structure
ModBase 3D Structure for Q04844
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.
Form/Format
Ascites, supplied as a liquid in PBS, pH 7.4, with 0.1% NaN3
Concentration
100ul (lot specific)
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.
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.
Related Product Information for
anti-AChR gamma antibody
Mouse monoclonal AChR gamma antibody
Product Categories/Family for anti-AChR gamma antibody
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
NCBI/Uniprot data below describe general gene information for AChR gamma. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
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
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]
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
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.

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Organs/Tissues associated with anti-AChR gamma antibody
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