上皮钠离子通道蛋白γ/γENaC抗体

英文名称    epithelial Sodium Channel gamma    

中文名称    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.    

说 明 书    0.1ml  0.2ml      

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    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 ICC=1:100-500 IF=1:100-500 上皮钠离子通道蛋白γ/γENaC抗体（石蜡切片需做抗原修复） 
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/1ml    

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 PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

上皮钠离子通道蛋白γ/γENaC抗体产品介绍    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.    

上皮钠离子通道蛋白γ/γENaC抗体

HZE784Hu 人膜联蛋白A4(ANXA4)ELISA试剂盒 ELISA Kit for Annexin A4 (ANXA4) 

HZF880Hu 人分泌型卷曲相关蛋白1(SFRP1)ELISA试剂盒 ELISA Kit for Secreted Frizzled Related Protein 1 (SFRP1) 

HZB097Hu 人运动因子相关蛋白1(MRP1)ELISA试剂盒 ELISA Kit for Motility Related Protein (MRP1) 

HZC102Hu 人骨成型蛋白3(BMP3)ELISA试剂盒 ELISA Kit for Bone Morphogenetic Protein 3 (BMP3) 

HZC797Hu 人非受体酪氨酸激酶2(TNK2)ELISA试剂盒 ELISA Kit for Tyrosine Kinase, Non Receptor 2 (TNK2) 

HZG685Hu 人肌钯蛋白(MYPN)ELISA试剂盒 ELISA Kit for Myopalladin (MYPN) 

CEG291Hu 人鸟苷酸环化酶激活因子2A(GUCA2A)ELISA试剂盒 ELISA Kit for Guanylate Cyclase Activator 2A (GUCA2A) 

HZL122Hu 人GM2神经节苷脂激活因子(GM2A)ELISA试剂盒 ELISA Kit for GM2 Ganglioside Activator (GM2A) 

HZD089Hu 人溶酶体酸性磷酸酶2(ACP2)ELISA试剂盒 ELISA Kit for Acid Phosphatase 2, Lysosomal (ACP2) 

HZB280Hu 人组织蛋白酶D(CTSD)ELISA试剂盒 ELISA Kit for Cathepsin D (CTSD) 

HZD058Hu 人中性鞘磷脂酶(NSMASE)ELISA试剂盒 ELISA Kit for Neutral Sphingomyelinase (NSMASE) 

HZA048Hu 人抑制素βE(INHβE)ELISA试剂盒 ELISA Kit for Inhibin Beta E (INHbE) 

HZL418Hu 人DEAD框肽5(DDX5)ELISA试剂盒 ELISA Kit for DEAD Box Polypeptide 5 (DDX5) 

HZD775Hu 人蛋白二硫化物异构酶A5(PDIA5)ELISA试剂盒 ELISA Kit for Protein Disulfide Isomerase A5 (PDIA5) 

HZH571Hu 人核小体装配蛋白1样1(NAP1L1)ELISA试剂盒 ELISA Kit for Nucleosome Assembly Protein 1 Like Protein 1 (NAP1L1) 

HZG324Hu 人妊娠带蛋白(PZP)ELISA试剂盒 ELISA Kit for Pregnancy Zone Protein (PZP) 

HZF102Hu 人精氨酸/丝氨酸丰富剪接因子2(SRSF2)ELISA试剂盒 ELISA Kit for Serine/Arginine Rich Splicing Factor 2 (SRSF2) 

HZE824Hu 人醛脱氢酶1家族成员A1(ALDH1A1)ELISA试剂盒 ELISA Kit for Aldehyde Dehydrogenase 1 Family, Member A1 (ALDH1A1) 

HZE174Hu 人前列腺蛋白类脂肪酶B(LIPB)ELISA试剂盒 ELISA Kit for Lipophilin B, Prostatein Like (LIPB) 

HZC598Hu 人血管非炎性蛋白1(VNN1)ELISA试剂盒 ELISA Kit for Vanin 1 (VNN1) 

HZD798Hu 人拓扑异构酶Ⅱβ(TOP2β)ELISA试剂盒 ELISA Kit for Topoisomerase II Beta (TOP2b) 

HZE676Hu 人再生胰岛衍生蛋白3γ(REG3γ)ELISA试剂盒 ELISA Kit for Regenerating Islet Derived Protein 3 Gamma (REG3g) 

HZF385Hu 人异连蛋白β(DTNβ)ELISA试剂盒 ELISA Kit for Dystrobrevin Beta (DTNb)