Key features and details
- Rabbit polyclonal to Nicotinic Acetylcholine Receptor alpha 1/CHRNA1
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 antibody
See all Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 primary antibodies
DescriptionRabbit polyclonal to Nicotinic Acetylcholine Receptor alpha 1/CHRNA1
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Synthetic peptide corresponding to Human Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 (internal sequence).
- Human prostate carcinoma tissue
This product was previously labelled as Nicotinic Acetylcholine Receptor alpha 1
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Storage bufferpH: 7.40
Preservative: 0.02% Sodium azide
Constituents: 0.88% Sodium chloride, 50% Glycerol
PBS without Mg2+ and Ca2+
Concentration information loading...
PurityImmunogen affinity purified
Purification notesThe antibody was affinity purified from Rabbit antiserum by affinity chromatography using epitope specific phosphopeptide. The antibody against non phosphopeptide was removed by chromatography using non phosphopeptide corresponding to the phosphorylation.
Our Abpromise guarantee covers the use of ab135272 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionAfter 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.
Tissue specificityIsoform 1 is only expressed in skeletal muscle. Isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.
Involvement in diseaseDefects in CHRNA1 are a cause of multiple pterygium syndrome lethal type (MUPSL) [MIM:253290]. Multiple pterygia are found infrequently in children with arthrogryposis and in fetuses with fetal akinesia syndrome. In lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia, and flexion contractures causing severe arthrogryposis and fetal akinesia. Subcutaneous edema can be severe, causing fetal hydrops with cystic hygroma and lung hypoplasia. Oligohydramnios and facial anomalies are frequent.
Note=The alpha subunit is the main focus for antibody binding in 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 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 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 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.
Sequence similaritiesBelongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Alpha-1/CHRNA1 sub-subfamily.
Cellular localizationCell junction > synapse > postsynaptic cell membrane. Cell membrane.
- Information by UniProt
- Acetylcholine receptor subunit alpha antibody
- ACHA_HUMAN antibody
- AChR antibody
ab135272 has not yet been referenced specifically in any publications.