Signal Transduction Antibodies 2

Anti-CHRNB1 Antibody (CAB5295)

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SKU:
CAB5295
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-CHRNB1 Antibody
Antibody SKU:CAB5295
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 25-235 of human CHRNB1 (NP_000738.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:Mouse skeletal muscle, Mouse heart, Mouse kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 25-235 of human CHRNB1 (NP_000738.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:EAEG RLRE KLFS GYDS SVRP AREV GDRV RVSV GLIL AQLI SLNE KDEE MSTK VYLD LEWT DYRL SWDP AEHD GIDS LRIT AESV WLPD VVLL NNND GNFD VALD ISVV VSSD GSVR WQPP GIYR SSCS IQVT YFPF DWQN CTMV FSSY SYDS SEVS LQTG LGPD GQGH QEIH IHEG TFIE NGQW EIIH KPSR LIQP PGDP RGGR EGQR QEV
Gene ID:1140
Uniprot:P11230
Cellular Location:Cell junction, Cell membrane, Multi-pass membrane protein, postsynaptic cell membrane, synapse
Calculated MW:49kDa/56kDa
Observed MW:63kDa
Synonyms:CHRNB1, ACHRB, CHRNB, CMS1D, CMS2A, CMS2C, SCCMS
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

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

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; acetylcholine binding; nicotinic acetylcholine-activated cation-selective channel activity; 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; synaptic transmission, cholinergic; neurological system process

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

NCBI Summary: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 Code:P11230
NCBI GenInfo Identifier:21903373
NCBI Gene ID:1140
NCBI Accession:P11230.3
UniProt Secondary Accession:P11230,Q8IZ46, Q96FB8, B7Z5H1,
UniProt Related Accession:P11230
Molecular Weight:501
NCBI Full Name:Acetylcholine receptor subunit beta
NCBI 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 Entry Name:ACHB_HUMAN
Anti-CHRNB1 Antibody (CAB5295)Western blot analysis of extracts of various cell lines, using CHRNB1 antibody (CAB5295) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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