ELISA/assay

Human Anti-Acetylcholine Receptor Antibody (Anti-AChR) ELISA Kit

MBS729942

48-Strip-Wells

470 USD

ELISA Kit

Human Anti-Acetylcholine Receptor Antibody (Anti-AChR) ELISA Kit

Acetylcholine Receptor Antibody (AChR)

Human Anti-Acetylcholine Receptor Antibody (Anti-AChR) ELISA Kit; Anti-Acetylcholine Receptor Antibody (Anti-AChR); Anti-Acetylcholine Receptor Antibody ELISA Kit (Anti-AChR) (Human)

acetylcholine receptor delta subunit; Acetylcholine receptor subunit delta; acetylcholine receptor subunit delta; acetylcholine receptor, nicotinic, delta (muscle); cholinergic receptor, nicotinic, delta polypeptide; cholinergic receptor, nicotinic, delta (muscle)

AChR

CHRND; CHRND; ACHRD; CMS2A; FCCMS; SCCMS; ACHRD

ACHD_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of AchRAb. No significant cross-reactivity or interference between AchRAb and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between AchRAb and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C

Samples: Serum, plasma, cell culture supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 0.1 ng/mL.

Human ELISA Kit

Intended Uses This AchRAb ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human AchRAb. This ELISA kit for research use only, not for therapeutic or diagnostic applications!. Principle of the Assay: AchRAb ELISA kit applies the competitive enzyme immunoassay technique utilizing Anti Acetylcholine Receptor antigen and an AchRAb-HRP conjugate. The assay sample and buffer are incubated together with AchRAb-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the AchRAb concentration since AchRAb from samples and AchRAb-HRP conjugate compete for the Anti Acetylcholine Receptor antigen binding site. Since the number of sites is limited, as more sites are occupied by AchRAb from the sample, fewer sites are left to bind AchRAb-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The AchRAb concentration in each sample is interpolated from this standard curve.

297402

CAA38759.1

Q07001

58,895 Da

Acetylcholine Binding And Downstream Events Pathway (106535); Activation Of Nicotinic Acetylcholine Receptors Pathway (161027); Highly Sodium Permeable Acetylcholine Nicotinic Receptors Pathway (161030); Neuroactive Ligand-receptor Interaction Pathway (83053); Neuroactive Ligand-receptor Interaction Pathway (462); Neuronal System Pathway (106513); Neurotransmitter Receptor Binding And Downstream Transmission In The Postsynaptic Cell Pathway (106534); Postsynaptic Nicotinic Acetylcholine Receptors Pathway (161031); Presynaptic Nicotinic Acetylcholine Receptors Pathway (161028); Transmission Across Chemical Synapses Pathway (106516)

The acetylcholine receptor of muscle has 5 subunits of 4 different types: 2 alpha and 1 each of beta, gamma and delta subunits. After acetylcholine binding, the receptor undergoes an extensive conformation change that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. Defects in this gene are a cause of multiple pterygium syndrome lethal type (MUPSL), congenital myasthenic syndrome slow-channel type (SCCMS), and congenital myasthenic syndrome fast-channel type (FCCMS). Several transcript variants, some protein-coding and some not, have been found for this gene. [provided by RefSeq, Feb 2012]

nAChRD: 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 CHRND 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. Defects in CHRND 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 CHRND 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. Delta/CHRND sub-subfamily. Protein type: Membrane protein, integral; Channel, ligand-gated; Membrane protein, multi-pass. Chromosomal Location of Human Ortholog: 2q37.1. Cellular Component: nicotinic acetylcholine-gated receptor-channel complex; postsynaptic membrane; plasma membrane; cell junction. Molecular Function: nicotinic acetylcholine-activated cation-selective channel activity; acetylcholine binding. Biological Process: skeletal muscle contraction; synaptic transmission; regulation of membrane potential; muscle contraction; transport; neuromuscular process; skeletal muscle growth; signal transduction; musculoskeletal movement. Disease: Myasthenic Syndrome, Congenital, 3a, Slow-channel; Myasthenic Syndrome, Congenital, 3c, Associated With Acetylcholine Receptor Deficiency; Multiple Pterygium Syndrome, Lethal Type; Myasthenic Syndrome, Congenital, 3b, Fast-channel

48-Strip-Wells

470 USD

96-Strip-Wells

675