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货号： bs-4263R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4263R-AF350
- 英文名称
- Anti-epithelial Sodium Channel gamma/AF350
- 中文名称
- AF350标记的上皮钠离子通道蛋白γ/γENaC抗体
- 别 名
- Amiloride sensitive epithelial sodium channel gamma subunit; Amiloride sensitive sodium channel subunit gamma; ENaC gamma subunit; ENaCg; ENaCgamma; Epithelial Na(+) channel subunit gamma; Epithelial Na+ channel subunit gamma; Gamma ENaC; Gamma NaCH; Nonvoltage gated sodium channel 1 subunit gamma; PHA 1; PHA1; SCNEG; SCNN 1G; SCNN1G; Sodium channel nonvoltage gated 1 gamma; SCNNG_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 免疫学 神经生物学 信号转导 通道蛋白 细胞膜受体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, 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
- 71kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human epithelial Sodium Channel gamma
- 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:
Epithelial sodium channels are amiloride-sensitive members of the Degenerin/epithelial sodium channel (Deg/ENaC) superfamily of ion channels. Members of this superfamily of ion channels share organizational similarity in that they all possess two short intracellular amino and carboxyl termini, two short membrane spanning segments, and a large extracellular loop with a conserved cysteine-rich region. There are three homologous isoforms of the ENaC (alpha, beta, and gamma) protein. ENaC in the kidney, lung, and colon plays an essential role in trans-epithelial sodium and fluid balance. ENaC also mediates aldosterone-dependent sodium reabsorption in the distal nephron of the kidney, thus regulating blood pressure. ENaC is thought to be regulated, in part, through association with the cystic fibrosis transmembrane conductance regulator (CFTR) chloride ion channel. Gain-of-function mutations in beta- or gamma-ENaC can cause severe arterial hypertension (Liddel’s syndrome) and loss-of-function mutations in alpha- or beta-ENaC causes pseudohypoaldosteronism (PHA-1).
Function:
Sodium permeable non-voltage-sensitive ion channel inhibited by the diuretic amiloride. Mediates the electrodiffusion of the luminal sodium (and water, which follows osmotically) through the apical membrane of epithelial cells. Controls the reabsorption of sodium in kidney, colon, lung and sweat glands. Also plays a role in taste perception.
Subunit:
Probable heterotrimer containing one alpha, one beta and one gamma subunit. A delta subunit can replace the alpha subunit. Interacts with the WW domains of NEDD4, NEDD4L, WWP1 and WWP2.
Subcellular Location:
Apical cell membrane; Multi-pass membrane protein. Note=Apical membrane of epithelial cells.
Post-translational modifications:
Phosphorylated on serine and threonine residues.
Ubiquitinated; this targets individual subunits for endocytosis and proteasome-mediated degradation.
DISEASE:
Defects in SCNN1G are a cause of Liddle syndrome (LIDDS) [MIM:177200]. It is an autosomal dominant disorder characterized by pseudoaldosteronism and hypertension associated with hypokalemic alkalosis. The disease is caused by constitutive activation of the renal epithelial sodium channel.
Defects in SCNN1G are the cause of bronchiectasis with or without elevated sweat chloride type 3 (BESC3) [MIM:613071]. A debilitating respiratory disease characterized by chronic, abnormal dilatation of the bronchi and other cystic fibrosis-like symptoms in the absence of known causes of bronchiectasis (cystic fibrosis, autoimmune diseases, ciliary dyskinesia, common variable immunodeficiency, foreign body obstruction). Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases.
Similarity:
Belongs to the amiloride-sensitive sodium channel (TC 1.A.6) family. SCNN1G subfamily.
Database links:Entrez Gene: 6340Human
Entrez Gene: 20278Mouse
Entrez Gene: 24768Rat
Omim: 600761Human
SwissProt: P51170Human
SwissProt: Q9WU39Mouse
SwissProt: Q28738Rabbit
SwissProt: P37091Rat
Unigene: 371727Human
Unigene: 35247Mouse
Unigene: 10360Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.