Target：AChRα1

Fields：Neuroactive ligand-receptor interaction

Gene Name：CHRNA1

Protein Name：Acetylcholine receptor subunit alpha

Human Gene Id：1134

Human Swiss Prot No：P02708

Mouse Gene Id：11435

Mouse Swiss Prot No：P04756

Rat Gene Id：79557

Rat Swiss Prot No：P25108

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.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. ELISA: 1:5000. IF 1:100-300 Not yet tested in other applications.

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：CHRNA1;ACHRA;CHNRA;Acetylcholine receptor subunit alpha

Observed Band(KD)：55kD

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

Subcellular Location：Cell junction, synapse, postsynaptic cell membrane ; Multi-pass membrane protein . Cell membrane ; Multi-pass membrane protein .

Expression：Isoform 1 is only expressed in skeletal muscle. Isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.