SKU: WP10919 Categories: ,

AChRβ1 Polyclonal Antibody

Price range: $268.00 through $328.00

SKU: WP10919-100
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Product details

Background:

The muscle acetylcholine receptor is composed of five subunits: two alpha subunits and one beta, one gamma, and one delta subunit. This gene encodes the beta subunit of the acetylcholine receptor. The acetylcholine receptor changes conformation upon acetylcholine binding leading to the opening of an ion-conducting channel across the plasma membrane. Mutations in this gene are associated with slow-channel congenital myasthenic syndrome. [provided by RefSeq, Jul 2008],

Specifications

TargetAChRβ1
ReactivityHuman, Mouse, Rat
ApplicationsWB, ELISA
MW(Calculated)
MW(Observed)55kD
Host SpeciesRabbit
IsotypeIgG
Conjugate/ModificationUnmodified
Modification
Recommended Dilution RatioWB 1:500-1:2000; ELISA 1:5000; Not yet tested in other applications.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration1 mg/ml
ClonalityPolyclonal
Clone Number
ImmunogenThe antiserum was produced against synthesized peptide derived from human CHRNB1. AA range:41-90
Sequence
SpecificityAChRβ1 Polyclonal Antibody detects endogenous levels of AChRβ1 protein.
Gene NameCHRNB1
Protein NameAcetylcholine receptor subunit beta
Other NameCHRNB1; / ACHRB; / CHRNB; / Acetylcholine receptor subunit beta
SpeciesHuman
Gene ID-11140
UniprotP11230,
Species.1Mouse
Gene ID-211443
Uniprot.1P09690,
Species.2Rat
Gene ID-324261
Uniprot.2P25109
Organism-4
Gene ID-4
SwissProt-4
BackgroundThe muscle acetylcholine receptor is composed of five subunits: two alpha subunits and one beta, one gamma, and one delta subunit. This gene encodes the beta subunit of the acetylcholine receptor. The acetylcholine receptor changes conformation upon acetylcholine binding leading to the opening of an ion-conducting channel across the plasma membrane. Mutations in this gene are associated with slow-channel congenital myasthenic syndrome. [provided by RefSeq, Jul 2008],
Cellular LocalizationCell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.
Tissue ExpressionEye,Muscle,
Signaling_pathway
Research Areas>>Neuroactive ligand-receptor interaction
FunctionDisease:Defects in CHRNB1 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.,Disease:Defects in CHRNB1 are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (ACHRDCMS) [MIM:608931]. ACHRDCMS is a post-synaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance.,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.,similarity:Belongs to the ligand-gated ionic channel (TC 1.A.9) family.,subunit:Pentamer of two alpha chains, and one each of the beta, delta, and gamma (in immature muscle) or epsilon (in mature muscle) chains.,
RRID
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