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SCNN1B Rabbit pAb (bs-4252R)  
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產品編號 bs-4252R
英文名稱 SCNN1B Rabbit pAb
中文名稱 上皮鈉通道β抗體
別    名 Amiloride sensitive sodium channel subunit beta; beta NaCH; ENaC beta; ENaCB; Epithelial Na(+) channel subunit beta; Epithelial Na+channel beta subunit; Epithelial Na+channel subunit beta; Epithelial sodium channel beta 2 subunit; Epithelial sodium channel beta 3 subunit; Nonvoltage gated sodium channel 1 beta subunit; SCNNB_HUMAN; Nonvoltage gated sodium channel 1 subunit beta; SCNEB; SCNN 1B; Sodium channel nonvoltage gated 1 beta(Liddle syndrome); Sodium channel nonvoltage gated 1 beta.  βENaC
Specific References  (2)     |     bs-4252R has been referenced in 2 publications.
[IF=2.586] Ay?e ?ak?r Gündo?du. et al. Impact of 5'-AMP-activated protein kinase (AMPK) on Epithelial Sodium Channels (ENaCs) in human sperm. TISSUE CELL. 2022 Aug;:101896  ELISA, IF ;  Human.  
[IF=2.586] Ay?e ?ak?r Gündo?du. et al. Resveratrol downregulates ENaCs through the activation of AMPK in human colon cancer cells. TISSUE CELL. 2023 Mar;:102071  ICC ;  Human.  
研究領域 腫瘤  神經生物學  信號轉導  細胞膜受體  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Horse)
產品應用 WB=1:500-2000,ICC/IF=1:100
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 73 kDa
檢測分子量 95-100/65-75
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCNN1B: 401-500/685 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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 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 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.

SWISS:
P51168

Gene ID:
6338

Database links:

Entrez Gene: 6338 Human

Entrez Gene: 20277 Mouse

Entrez Gene: 24767 Rat

Omim: 600760 Human

SwissProt: P51168 Human

SwissProt: Q9WU38 Mouse

SwissProt: P37090 Rat

Unigene: 414614 Human

Unigene: 7709 Mouse

Unigene: 9807 Rat



產品圖片
Sample: Lane 1: Human A673 cell lysates Primary: Anti-SCNN1B (bs-4252R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 73 kDa Observed band size: 110 kDa
U266 cell; 4% Paraformaldehyde-fixed; Triton X-100 at room temperature for 20 min; Blocking buffer (normal goat serum, C-0005) at 37°C for 20 min; Antibody incubation with (SCNN1B) polyclonal Antibody, Unconjugated (bs-4252R) 1:100, 90 minutes at 37°C; followed by a conjugated Goat Anti-Rabbit IgG antibody at 37°C for 90 minutes, DAPI (blue, C02-04002) was used to stain the cell nuclei.
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