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AChRα1 Polyclonal Antibody

Polyclonal antibody

Specification

BYab-16527

  • 50UL $180 100UL $255
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Host
Reactiveness
Use
Molecular weight (DA)
55kD
Immunogen
The antiserum was produced against synthesized peptide derived from the Internal region of human CHRNA1. AA range:171-220
Specificity
AChRα1 Polyclonal Antibody detects endogenous levels of AChRα1 protein.
Source
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/5000. IF 1:100-300 Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
1 mg/ml
Background
The muscle acetylcholine receptor consiststs of 5 subunits of 4 different types: 2 alpha subunits and 1 each of the beta, gamma, and delta subunits. This gene encodes an alpha subunit that plays a role in acetlycholine binding/channel gating. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Nov 2012],
Function
disease: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.,disease: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
Gene Name
CHRNA1
Protein name
Acetylcholine receptor subunit alpha
Abbreviation
AChRα1
Other name
CHRNA1; ACHRA; CHNRA; Acetylcholine receptor subunit alpha
Fields
>>Neuroactive ligand-receptor interaction
Human gene ID
1134
Human protein sequence Database
P02708
Mouse gene ID
11435
Mouse protein sequence database
P04756
Rat gene ID
79557
Rat protein sequence database
P25108
Cellular localization
Cell junction, synapse, postsynaptic cell membrane ; Multi-pass membrane protein . Cell membrane ; Multi-pass membrane protein .
Tissue expression
Isoform 1 is only expressed in skeletal muscle. Isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.
Storage
-20°C/1 year

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AChRα1 Polyclonal Antibody

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