Mouse anti-Human AChR beta Monoclonal Antibody | anti-CHRNB1 antibody

AChR beta (Acetylcholine Receptor Subunit beta) (PE)

Cat. Num. AAA6251332

• Gene Names: CHRNB1; ACHRB; CHRNB; CMS1D; CMS2A; SCCMS
• Reactivity: Human
• Applications: Flow Cytometry, Functional Assay, Immunohistochemistry, Immunoprecipitation, Western Blot
• Purity: Purified by protein A affinity chromatography from tissue culture supernatant.
• Synonyms: AChR beta; Monoclonal Antibody; AChR beta (Acetylcholine Receptor Subunit beta) (PE); anti-CHRNB1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: B3
• Specificity: Recognizes human AChR beta.
• Purity/Purification: Purified by protein A affinity chromatography from tissue culture supernatant.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-CHRNB1 antibody: Flow Cytometry (FC/FACS), Immunohistochemistry (IHC), Immunoprecipitation (IP), Western Blot (WB)
• Immunogen: Human Acetylcholine Receptor (AChR).
• Conjugate: PE
• Preparation and Storage: Store at 4 degree C. Do Not Freeze.

Testing Data

(A lysate overexpressing acteylcholine receptor beta probed with Mouse anti Human acetylcholine receptor beta followed by Rabbit F(ab')2 anti Mouse IgG:HRP)

Product Categories/Family for anti-CHRNB1 antibody

Antibodies; Receptors

References

1. Whiting, P.J. et al. (1986) Monoclonal Antibodies that distinguish between normal and denervated human acetylcholine receptor. J. Neuroimmunol. 11: 223-235. 2. Roberts, A. et al. (1992) Search for cross reactive idiotypes on monoclonal and myasthenia gravis acetylcholine receptor antibodies. Autoimmunity 21: 53-60. 3. Heidenreich F. et al. (1988) Differences in fine specificity of anti-acetylcholine receptor antibodies between subgroups of spontaneous myasthenia gravis of recent onset and of penicillamine induced myasthenia. Autoimmunity 2: 31-37. 4. Whiting, P. et al. (1986) Myasthenia gravis: Monoclonal anti-human acetylcholine receptor antibodies used to analyse antibody specificities and responses to treatment. Neurology 36: 612-617. 5. Schluep, M. et al. (1987) Acetylcholine receptors in human thymic myoid cells in situ: An immunohistological study. Annals of Neurology 22: 212-222.

NCBI and Uniprot Product Information

• NCBI GI #: 41327726
• NCBI GeneID: 1140
• NCBI Accession #: NP_000738.2
• NCBI GenBank Nucleotide #: NM_000747.2
• UniProt Accession #: P11230; Q96FB8
• Molecular Weight: 56,698 Da
• NCBI Official Full Name: acetylcholine receptor subunit beta
• NCBI Official Synonym Full Names: cholinergic receptor, nicotinic, beta 1 (muscle)
• NCBI Official Symbol: CHRNB1
• NCBI Official Synonym Symbols: ACHRB; CHRNB; CMS1D; CMS2A; SCCMS
• NCBI Protein Information: acetylcholine receptor subunit beta; acetylcholine receptor, nicotinic, beta 1 (muscle); cholinergic receptor, nicotinic, beta polypeptide 1 (muscle)
• UniProt Protein Name: Acetylcholine receptor subunit beta
• Protein Family: Acetylcholine receptor
• UniProt Gene Name: CHRNB1
• UniProt Synonym Gene Names: ACHRB; CHRNB
• UniProt Entry Name: ACHB_HUMAN

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: Channel, cation; Membrane protein, integral; Membrane protein, multi-pass; Channel, ligand-gated

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; neuromuscular synaptic transmission; behavioral response to nicotine; muscle fiber development; postsynaptic membrane organization; signal transduction; transmembrane transport; cation transport; synaptic transmission, cholinergic; neurological system process

Disease: Myasthenic Syndrome, Congenital, 2c, Associated With Acetylcholine Receptor Deficiency; Myasthenic Syndrome, Congenital, 2a, Slow-channel