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Rabbit Anti-Alpha-ENaC/FITC Conjugated antibody (bs-2957R-FITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2957R-FITC
英文名稱1 Rabbit Anti-Alpha-ENaC/FITC Conjugated antibody
中文名稱 FITC標記的鈉通道蛋白α 抗體
別    名 Alpha ENaC 2; Alpha ENaC; Alpha NaCH; Alpha-ENaC; Alpha-NaCH; Amiloride sensitive epithelial sodium channel alpha subunit; Amiloride sensitive sodium channel subunit alpha; Amiloride-sensitive sodium channel subunit alpha; ENaCa; ENaCalpha; Epithelial Na(+) channel subunit alpha; Epithelial Na+ channel subunit alpha; FLJ21883; Nonvoltage gated sodium channel 1 subunit alpha; Nonvoltage-gated sodium channel 1 subunit alpha; SCNEA; SCNN 1; SCNN1; SCNN1A; SCNNA_HUMAN; Sodium channel nonvoltage gated 1 alpha.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 神經生物學  信號轉導  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Dog, Pig, Cow, Horse, Sheep, )
產品應用 Flow-Cyt=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 76kDa
細胞定位 細胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Alpha-ENaC
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 background:
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.

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.

Tissue Specificity:
Highly expressed in kidney and lung. Detected at intermediate levels in pancreas and liver, and at low levels in heart and placenta. Isoform 1 and isoform 2 predominate in all tissues. Expression of isoform 3, isoform 4 and isoform 5 is very low or not detectable, except in lung and heart.

Post-translational modifications:
Ubiquitinated; this targets individual subunits for endocytosis and proteasome-mediated degradation (By similarity).
ENaC cleavage by furin, and subsequently by prostasin (PRSS8), leads to a stepwise increase in the open probability of the channel as a result of release of the alpha and gamma subunit inhibitory tracts, respectively.

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).
Bronchiectasis with or without elevated sweat chloride 2 (BESC2) [MIM:613021]: 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. SCNN1A subfamily.

Database links:

Entrez Gene: 6337 Human

Entrez Gene: 20276 Mouse

Entrez Gene: 25122 Rat

Omim: 600228 Human

SwissProt: P37088 Human

SwissProt: Q61180 Mouse

SwissProt: P37089 Rat

Unigene: 591047 Human

Unigene: 144114 Mouse

Unigene: 9808 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

αENaC
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