Signal Transduction Antibodies 3

Anti-CHRNG Antibody (CAB7884)

(No reviews yet) Write a Review
SKU:
CAB7884
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-CHRNG Antibody
Antibody SKU:CAB7884
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 328-517 of human CHRNG (NP_005190.4).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse skeletal muscle, Rat skeletal muscle
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 328-517 of human CHRNG (NP_005190.4).
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:NVSL RSPH THSM ARGV RKVF LRLL PQLL RMHV RPLA PAAV QDTQ SRLQ NGSS GWSI TTGE EVAL CLPR SELL FQQW QRQG LVAA ALEK LEKG PELG LSQF CGSL KQAA PAIQ ACVE ACNL IACA RHQQ SHFD NGNE EWFL VGRV LDRV CFLA MLSL FICG TAGI FLMA HYNR VPAL PFPG DPRP YLPS PD
Gene ID:1146
Uniprot:P07510
Cellular Location:Cell junction, Cell membrane, Multi-pass membrane protein, postsynaptic cell membrane, synapse
Calculated MW:52kDa/57kDa
Observed MW:60KDa
Synonyms:CHRNG, ACHRG
Background:The mammalian muscle-type acetylcholine receptor is a transmembrane pentameric glycoprotein with two alpha subunits, one beta, one delta, and one epsilon (in adult skeletal muscle) or gamma (in fetal and denervated muscle) subunit. This gene, which encodes the gamma subunit, is expressed prior to the thirty-third week of gestation in humans. The gamma subunit of the acetylcholine receptor plays a role in neuromuscular organogenesis and ligand binding and disruption of gamma subunit expression prevents the correct localization of the receptor in cell membranes. Mutations in this gene cause Escobar syndrome and a lethal form of multiple pterygium syndrome. Muscle-type acetylcholine receptor is the major antigen in the autoimmune disease myasthenia gravis.
UniProt Protein Function:nAChRG: 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 CHRNG 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 CHRNG are a cause of multiple pterygium syndrome Escobar variant (MUPSE); also known as nonlethal type multiple pterygium syndrome. Escobar syndrome is a non-lethal form of arthrogryposis multiplex congenita. It is an autosomal recessive condition characterized by excessive webbing (pterygia), congenital contractures (arthrogryposis), and scoliosis. Variable other features include intrauterine death, congenital respiratory distress, short stature, faciocranial dysmorphism, ptosis, low-set ears, arachnodactyly and cryptorchism in males. Congenital contractures are common and may be caused by reduced fetal movements at sensitive times of development. Possible causes of decreased fetal mobility include space constraints such as oligohydramnion, drugs, metabolic conditions or neuromuscular disorders including myasthenia gravis. is a. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Gamma/CHRNG sub-subfamily.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 2q37.1

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

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

Biological Process: muscle contraction; neuromuscular synaptic transmission; response to nicotine; signal transduction; synaptic transmission, cholinergic; transport

Disease: Multiple Pterygium Syndrome, Escobar Variant; Multiple Pterygium Syndrome, Lethal Type

NCBI Summary:The mammalian muscle-type acetylcholine receptor is a transmembrane pentameric glycoprotein with two alpha subunits, one beta, one delta, and one epsilon (in adult skeletal muscle) or gamma (in fetal and denervated muscle) subunit. This gene, which encodes the gamma subunit, is expressed prior to the thirty-third week of gestation in humans. The gamma subunit of the acetylcholine receptor plays a role in neuromuscular organogenesis and ligand binding and disruption of gamma subunit expression prevents the correct localization of the receptor in cell membranes. Mutations in this gene cause Escobar syndrome and a lethal form of multiple pterygium syndrome. Muscle-type acetylcholine receptor is the major antigen in the autoimmune disease myasthenia gravis.[provided by RefSeq, Sep 2009]
UniProt Code:P07510
NCBI GenInfo Identifier:126302510
NCBI Gene ID:1146
NCBI Accession:P07510.2
UniProt Secondary Accession:P07510,Q14DU4, Q53RG2, B3KWM8,
UniProt Related Accession:P07510
Molecular Weight:52,020 Da
NCBI Full Name:Acetylcholine receptor subunit gamma
NCBI Synonym Full Names:cholinergic receptor nicotinic gamma subunit
NCBI Official Symbol:CHRNG  
NCBI Official Synonym Symbols:ACHRG  
NCBI Protein Information:acetylcholine receptor subunit gamma
UniProt Protein Name:Acetylcholine receptor subunit gamma
Protein Family:Acetylcholine receptor
UniProt Gene Name:CHRNG  
UniProt Entry Name:ACHG_HUMAN
Anti-CHRNG Antibody (CAB7884)Western blot analysis of extracts of various cell lines, using CHRNG antibody (CAB7884) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10001 dilution. Lysates/proteins: 26ug per lane. Blocking buffer: 4% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 3s.
View AllClose

0 Reviews

View AllClose

Related Products