ITT0084
ITT0084
- Catalog: ITT0084
- Gene/Protein: CHRNB1
- Product Description: Immunotag™ AChRβ1 Polyclonal Antibody
385.0000
Price in reward points: 385
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Antibody Specification | |
Datasheet | |
Target Protein | AChRβ1 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Mouse,Rat |
Host Species | Rabbit |
Immunogen | Synthesized peptide derived from AChRβ1, at AA range: 10-90 |
Specificity | AChRβ1 Polyclonal Antibody detects endogenous levels of AChRβ1 protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | CHRNB1 |
Accession No. | P11230 P09690 P25109 |
Alternate Names | CHRNB1; ACHRB; CHRNB; Acetylcholine receptor subunit beta |
Description | cholinergic receptor nicotinic beta 1 subunit(CHRNB1) Homo sapiens 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], |
Protein Expression | Eye,Muscle, |
Subcellular Localization | integral component of plasma membrane,acetylcholine-gated channel complex,integral component of membrane,cell junction,synapse,postsynaptic membrane, |
Protein Function | disease: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., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |