Signal Transduction Antibodies 1

Anti-CHRNE Antibody (CAB10057)

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SKU:
CAB10057
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-CHRNE Antibody
Antibody SKU:CAB10057
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 21-239 of human CHRNE (NP_000071.1).
Application:WB
Recommended Dilution:WB 1:1000 - 1:4000
Reactivity:Human, Mouse, Rat
Positive Samples:HepG2, Mouse skeletal muscle, Rat kidney, Rat skeletal muscle
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 21-239 of human CHRNE (NP_000071.1).
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:KNEE LRLY HHLF NNYD PGSR PVRE PEDT VTIS LKVT LTNL ISLN EKEE TLTT SVWI GIDW QDYR LNYS KDDF GGIE TLRV PSEL VWLP EIVL ENNI DGQF GVAY DANV LVYE GGSV TWLP PAIY RSVC AVEV TYFP FDWQ NCSL IFRS QTYN AEEV EFTF AVDN DGKT INKI DIDT EAYT ENGE WAID FCPG VIRR HHGG ATDG PGET DVIY SLII RRK
Gene ID:1145
Uniprot:Q04844
Cellular Location:Cell junction, Cell membrane, Multi-pass membrane protein, postsynaptic cell membrane, synapse
Calculated MW:54kDa
Observed MW:45kDa
Synonyms:CHRNE, ACHRE, CMS1D, CMS1E, CMS2A, CMS4A, CMS4B, CMS4C, FCCMS, SCCMS
Background: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.
UniProt Protein Function:nAChRE: 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. 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. Defects in CHRNE 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 CHRNE 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. Defects in CHRNE 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. Epsilon/CHRNE sub-subfamily.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 17p13.2

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

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

Biological Process: muscle contraction; neuromuscular synaptic transmission; regulation of membrane potential; response to nicotine; signal transduction; skeletal muscle contraction; synaptic transmission; synaptic transmission, cholinergic; transport

Disease: Myasthenic Syndrome, Congenital, 4a, Slow-channel; Myasthenic Syndrome, Congenital, 4b, Fast-channel; Myasthenic Syndrome, Congenital, Associated With Acetylcholine Receptor Deficiency

NCBI Summary: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]
UniProt Code:Q04844
NCBI GenInfo Identifier:4557463
NCBI Gene ID:1145
NCBI Accession:NP_000071.1
UniProt Secondary Accession:Q04844,D3DTK6,
UniProt Related Accession:Q04844
Molecular Weight:Calculated MW: 54kDaMolecular Weight: 45kDa
NCBI Full Name:acetylcholine receptor subunit epsilon
NCBI Synonym Full Names:cholinergic receptor nicotinic epsilon subunit
NCBI Official Symbol:CHRNE  
NCBI Official Synonym Symbols:ACHRE; CMS1D; CMS1E; CMS2A; CMS4A; CMS4B; CMS4C; FCCMS; SCCMS  
NCBI Protein Information:acetylcholine receptor subunit epsilon
UniProt Protein Name:Acetylcholine receptor subunit epsilon
Protein Family:Acetylcholine receptor
UniProt Gene Name:CHRNE  
UniProt Entry Name:ACHE_HUMAN
Anti-CHRNE Antibody (CAB10057)Western blot analysis of extracts of various cell lines, using CHRNE antibody (CAB10057) 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: 1s.
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