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货号： bs-19245R-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19245R-Cy3
- 英文名称
- Anti-CHRNG/Cy3
- 中文名称
- Cy3标记的烟碱型乙酰胆碱受体γ抗体
- 别 名
- Acetylcholine receptor muscle gamma subunit;Nicotinic Acetylcholine Receptor gamma; Acetylcholine receptor protein gamma chain precursor; Acetylcholine receptor subunit gamma; ACHG; ACHG_HUMAN; Achr 3; Achr3; ACHRG; ACRG; Cholinergic receptor nicotinic gamma; Cholinergic receptor nicotinic gamma polypeptide; CHRNG; MGC133376.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 神经生物学 细胞膜受体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 55kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CHRNG
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- 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.[provided by RefSeq, Sep 2009]
Function:
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.
Subcellular Location:
Cell junction; synapse; postsynaptic cell membrane. Cell membrane.
DISEASE:
Defects in CHRNG are a cause of multiple pterygium syndrome lethal type (MUPSL) [MIM:253290]. 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) [MIM:265000]; 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.
Similarity:
Belongs to the ligand-gated ion channel (TC 1.A.9) family.
Acetylcholine receptor (TC 1.A.9.1) subfamily.
Gamma/CHRNG sub-subfamily.
Database links:Entrez Gene: 1146Human
Omim: 100730Human
SwissProt: P07510Human
Unigene: 248101Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.