Mouse AChR BETA Monoclonal Antibody | anti-CHRNB1 antibody
MOUSE ANTI HUMAN ACETYLCHOLINE RECEPTOR BETA
Purified IgG - liquid
Shelf Life: 18 months from the date of despatch
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
nAChRB1: 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. Defects in CHRNB1 are a cause of congenital myasthenic syndrome slow-channel type (SCCMS). 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 CHRNB1 are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (CMS-ACHRD). CMS-ACHRD is a postsynaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Beta- 1/CHRNB1 sub-subfamily.
Protein type: Membrane protein, multi-pass; Channel, cation; Channel, ligand-gated; Membrane protein, integral
Chromosomal Location of Human Ortholog: 17p13.1
Cellular Component: nicotinic acetylcholine-gated receptor-channel complex; postsynaptic membrane; integral to plasma membrane; synapse; cell junction
Molecular Function: channel activity; acetylcholine receptor activity; nicotinic acetylcholine-activated cation-selective channel activity; acetylcholine binding; ligand-gated ion channel activity
Biological Process: skeletal muscle contraction; regulation of membrane potential; muscle contraction; behavioral response to nicotine; neuromuscular synaptic transmission; muscle fiber development; signal transduction; postsynaptic membrane organization; transmembrane transport; cation transport; neurological system process; synaptic transmission, cholinergic
Disease: Myasthenic Syndrome, Congenital, 2c, Associated With Acetylcholine Receptor Deficiency; Myasthenic Syndrome, Congenital, 2a, Slow-channel
Research Articles on CHRNB1
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Product Notes
The CHRNB1 chrnb1 (Catalog #AAA219929) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's AChR BETA can be used in a range of immunoassay formats including, but not limited to, Immunohistology Frozen, Flow cytometry (FC/FACS), Immunoprecipitation (IP), Western Blot (WB). Western Blotting: Minimum Dilution: 1/100; Maximum Dilution: 1/1000. Researchers should empirically determine the suitability of the CHRNB1 chrnb1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "AChR BETA, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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