磷酸化上皮钠通道β2抗体
规格：1mg/1ml
英文名： phospho-SCNN1B(Thr615)
别名： SCNN1B(phospho T615); Amiloride sensitive sodium channel subunit beta; Beta NaCH; ENaC beta; ENaCB; Epithelial Na(+) channel subunit beta; Epithelial Na+ channel beta subunit; Epithelial Na+ channel s
分子量： 73kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated Synthesised phosphopeptide derived from human
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Guinea Pig,
细胞定位：
磷酸化上皮钠通道β2抗体产品介绍：background: SCNN1B is a subunit of the epithelial sodium channel, ENaC. ENac has high sodium selectivity, low conductance, and amiloride sensitivity. The functional channel of ENaC is composed of at least 3 subunits, alpha (SCNN1A), beta (SCNN1B), and gamma (SCNN1G). The 3 subunits show sequence similarities to one another, indicating descent from a common ancestral gene. Each encodes a protein containing 2 transmembrane domains, with intracellular amino and carboxyl termini. 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. Interacts with the full length immature form of PCSK9 (pro-PCSK9). Subcellular Location: Apical cell membrane; Multi-pass membrane protein. Note=Apical membrane of epithelial cells. Post-translational modifications: Phosphorylated on serine and threonine residues (By similarity). DISEASE: Pseudohypoaldosteronism 1, autosomal recessive (PHA1B) [MIM:264350]: A rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. PHA1B is a severe form involving multiple磷酸化上皮钠通道β2抗体 organ systems, and characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatremia, hyperkalemia, metabolic acidosis, failure to thrive and weight loss. Note=The disease is caused by mutations affecting the gene represented in this entry. The degree of channel function impairment differentially affects the renin-aldosterone system and urinary Na/K ratios, resulting in distinct genotype-phenotype relationships in PHA1 patients. Loss-of-function mutations are associated with a severe clinical course and age-dependent hyperactivation of the renin-aldosterone system. This feature is not observed in patients with missense mutations that reduce but do not eliminate channel function. Markedly reduced channel activity results in impaired linear growth and delayed puberty (PubMed:18634878). Liddle syndrome (LIDDS) [MIM:177200]: Autosomal dominant disorder characterized by pseudoaldosteronism and hypertension associated with hypokalemic alkalosis. The disease is caused by constitutive 磷酸化上皮钠通道β2抗体activation of the renal epithelial sodium channel. Note=The disease is caused by mutations affecting the gene represented in this entry. Bronchiectasis with or without elevated sweat chloride 1 (BESC1) [MIM:211400]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the amiloride-sensitive sodium channel (TC 1.A.6) family. SCNN1B subfamily. Database links: UniProtKB/Swiss-Prot: P51168.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
磷酸化上皮钠通道β2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （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.
研究领域：肿瘤 **学 神经生物学 通道蛋白 细胞表面分子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid