Rabbit AChRalpha1 Polyclonal Antibody | anti-AChRalpha1 antibody

AChRalpha1 (E217) Polyclonal Antibody

Cat. Num. AAA3003120

• Gene Names: CHRNA1; ACHRA; ACHRD; CHRNA; CMS1A; CMS1B; CMS2A; FCCMS; SCCMS
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: AChRalpha1; Polyclonal Antibody; AChRalpha1 (E217) Polyclonal Antibody; Acetylcholine receptor subunit alpha; ACHRA; CHNRA; CHRNA1; ACHRD; CMS2A; FCCMS; SCCMS; Acra; anti-AChRalpha1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: AChRalpha1 (E217) polyclonal antibody detects endogenous levels of AChRalpha1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 482

• Applicable Applications for anti-AChRalpha1 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 181-230 of Human AChRalpha1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of AChRalpha1 (E217) pAb at 1:500 dilutionLane1:HepG2 whole cell lysate (40ug) Lane2:A549 whole cell lysate (40ug) Lane3:PC3 whole cell lysate (40ug) Lane4:CT26 whole cell lysate (40ug) Lane5:H9C2 whole cell lysate (40ug))

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of AChRalpha1 (E217) pAb bin paraffin-embedded human liver carcinoma tissue at 1:50,showing membrnae and cytoplasmic staining. Negative control (the right) Using PBS instead of primary antibody, secondary antibody is Goat Anti-Rabbit IgG-biotin followed by avidin-peroxidase.)

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of AChRalpha1 (E217) pAb bin paraffin-embedded human kidney carcinoma tissue at 1:50,showing membrnae and cytoplasmic staining. Negative control (the right) Using PBS instead of primary antibody, secondary antibody is Goat Anti-Rabbit IgG-biotin followed by avidin-peroxidase.)

Related Product Information for anti-AChRalpha1 antibody

Nicotinic acetylcholine receptors(AChRs) are found at the postsynaptic membrane of the neuromuscular junctionand bind acetylcholine molecules, allowing ions to move through thepore. Glutamate receptors are found in the postsynaptic membrane of cellsin the central nervous system. AChRalpha1, also known as ACHRD, CHRNA, CMS2A, FCCMS, SCCMS or CHRNA1, is a 482 amino acid multi-pass membrane protein that exists as two alternatively spliced isoforms, which are expressed in different tissues. Isoform 1 is only expressed in skeletal muscle whereas isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus.

NCBI and Uniprot Product Information

• NCBI GI #: 87567783
• NCBI GeneID: 1134
• NCBI Accession #: NP_001034612.1
• NCBI GenBank Nucleotide #: NP_001034612.1
• UniProt Accession #: P02708
• Molecular Weight: ~ 55kDa
• NCBI Official Full Name: acetylcholine receptor subunit alpha isoform a
• NCBI Official Synonym Full Names: cholinergic receptor nicotinic alpha 1 subunit
• NCBI Official Symbol: CHRNA1
• NCBI Official Synonym Symbols: ACHRA; ACHRD; CHRNA; CMS1A; CMS1B; CMS2A; FCCMS; SCCMS
• NCBI Protein Information: acetylcholine receptor subunit alpha
• UniProt Protein Name: Acetylcholine receptor subunit alpha
• UniProt Gene Name: CHRNA1
• UniProt Synonym Gene Names: ACHRA; CHNRA
• UniProt Entry Name: ACHA_HUMAN

NCBI Description

The muscle acetylcholine receptor consiststs of 5 subunits of 4 different types: 2 alpha subunits and 1 each of the beta, gamma, and delta subunits. This gene encodes an alpha subunit that plays a role in acetlycholine binding/channel gating. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Nov 2012]

Uniprot Description

nAChRA1: 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 CHRNA1 are a cause of multiple pterygium syndrome lethal type (MUPSL). 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. 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). 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). 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. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Alpha- 1/CHRNA1 sub-subfamily. 2 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 2q31.1

Cellular Component: nicotinic acetylcholine-gated receptor-channel complex; postsynaptic membrane; cell surface; plasma membrane; neuromuscular junction; cell junction

Molecular Function: acetylcholine receptor activity; ion channel activity; acetylcholine binding; nicotinic acetylcholine-activated cation-selective channel activity

Biological Process: muscle maintenance; skeletal muscle contraction; synaptic transmission; regulation of membrane potential; transport; neuromuscular process; generation of action potential; neuromuscular synaptic transmission; skeletal muscle growth; musculoskeletal movement; signal transduction; neuromuscular junction development

Disease: Myasthenic Syndrome, Congenital, Fast-channel; Myasthenic Syndrome, Congenital, Slow-channel; Multiple Pterygium Syndrome, Lethal Type