Mouse Anti-CHRNE Recombinant Antibody (C7988) (CBMAB-C7988-LY)

Mouse

Human, Mouse, Rat

C7988

IgG1, κ

WB, IP, IF, ELISA

Amino acids 334-441 mapping at the C-terminus of AChRε of human.

Mouse

Human, Mouse, Rat

IgG1, κ

Monoclonal Antibody

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500
ELISA	1:100-1:1,000

Liquid

PBS, 0.1% gelatin

< 0.1% sodium azide

0.2 mg/ml

> 95% Purity determined by SDS-PAGE.

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

cholinergic receptor, nicotinic, epsilon

Acetylcholine receptors at mature mammalian neuromuscular junctions are pentameric protein complexes composed of four subunits in the ratio of two alpha subunits to one beta, one epsilon, and one delta subunit. The acetylcholine receptor changes subunit composition shortly after birth when the epsilon subunit replaces the gamma subunit seen in embryonic receptors. Mutations in the epsilon subunit are associated with congenital myasthenic syndrome. [provided by RefSeq, Sep 2009]

Cholinergic Receptor Nicotinic Epsilon Subunit; Acetylcholine Receptor, Nicotinic, Epsilon (Muscle); Cholinergic Receptor, Nicotinic, Epsilon (Muscle); Cholinergic Receptor, Nicotinic Epsilon; ACHRE; Cholinergic Receptor, Nicotinic, Epsilon Polypeptide; C

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.

Chemical synaptic transmission Source: GO_Central
Ion transmembrane transport Source: GO_Central
Muscle contraction Source: ProtInc
Nervous system process Source: GO_Central
Regulation of membrane potential Source: GO_Central
Signal transduction Source: GO_Central
Synaptic transmission, cholinergic Source: ProtInc

Postsynaptic cell membrane; Cell membrane

The muscle AChR is the major target antigen in the autoimmune disease 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.
Myasthenic syndrome, congenital, 4A, slow-channel (CMS4A): A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and 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. CMS4A is a slow-channel myasthenic syndrome. It is caused by kinetic abnormalities of the AChR, resulting in prolonged AChR channel opening episodes, prolonged endplate currents, and depolarization block. This is associated with calcium overload, which may contribute to subsequent degeneration of the endplate and postsynaptic membrane.
Myasthenic syndrome, congenital, 4B, fast-channel (CMS4B): A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and 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. CMS4B is a fast-channel myasthenic syndrome. It is caused by kinetic abnormalities of the AChR, resulting in brief opening and activity of the channel, with a rapid decay in endplate current, failure to achieve threshold depolarization of the endplate and consequent failure to fire an action potential.
Myasthenic syndrome, congenital, 4C, associated with acetylcholine receptor deficiency (CMS4C): A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and 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. CMS4C is an autosomal recessive disorder of postsynaptic neuromuscular transmission, due to deficiency of AChR at the endplate that results in low amplitude of the miniature endplate potential and current.

Extracellular: 21-239
Helical: 240-264
Cytoplasmic: 265-272
Helical: 273-291
Extracellular: 292-306
Helical: 307-328
Cytoplasmic: 329-456
Helical: 457-480
Extracellular: 481-493