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D-氨基半乳糖鹽酸鹽號：1772-03-8   
英文名稱：D-Galactosamine HC1；2-Amino-2-deoxy-D-galactose hydrochloride；D-Chondrosamine hydrochloride    
其他名稱：D-半乳糖胺鹽酸鹽；2-氨基-2-脫氧-D-半乳糖鹽酸鹽；D-軟骨糖胺鹽酸鹽；2-氨基-2-脫氧-D-水解乳糖鹽酸鹽   
號：1772-03-8    
C6H13NO5?HCl=215.63   
級別：試劑級   
含量：≥98.0%   
干燥失重：≤1.0%   
性狀(以下信息僅供參考)：白色或類白色結(jié)晶性粉末   
用途：本品僅供科研，不得用于其它用途。   
保存：RT 客戶根據(jù)D-氨基半乳糖鹽酸鹽號：1772-03-8 性質(zhì)、化學式、分子式、結(jié)構(gòu)式、比重、密度、號、沸點、熔點、水溶性、MSDS、用途、作用、規(guī)格包裝、性狀、注意事項、英文名、別稱、純度、級別等情況，本產(chǎn)品化學性質(zhì)穩(wěn)定，運輸條件不苛刻，一般儲存在陰涼，干燥，通風良好的地方，遠離不相容的物質(zhì)。保持容器密閉。
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 optimal dilutions/concentrations should be determined by the end user. 
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.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)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.
Database links : UniProtKB/Swiss-Prot: P51168.2
英文名稱  Anti-phospho-SCNN1B(Thr615)
中文名稱  磷酸化上皮鈉通道β2抗體
別    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 subunit beta; Epithelial sodium channel beta 2 subunit; Epithelial sodium channel beta 3 subunit; Nonvoltage gated sodium channel 1 beta subunit; Nonvoltage gated sodium channel 1 subunit beta; SCNEB; SCNN 1B; SCNNB_HUMAN; Sodium channel nonvoltage gated 1 beta (Liddle syndrome); Sodium channel nonvoltage gated 1 beta.
濃    度  1mg/1ml
規(guī) 格  0.1ml/100μg
抗體來源  Rabbit 
克隆類型  polyclonal
交叉反應  Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Guinea Pig
產(chǎn)品類型  一抗  磷酸化抗體  
研究領(lǐng)域  腫瘤 免疫學 神經(jīng)生物學 通道蛋白 細胞表面分子 
蛋白分子量  predicted molecular weight: 73kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated Synthesised phosphopeptide derived from human SCNN1B around the phosphorylation site of Thr615
亞    型  IgG
純化方法  affinity purified by Protein A
儲 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
產(chǎn)品應用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  Flow-Cyt=1:100-500  IF=1:100-500
（石蠟切片需做抗原修復）
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user. 
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.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)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.
Database links : UniProtKB/Swiss-Prot: P51168.2
英文名稱  Anti-phospho-SCNN1B(Ser633) 
中文名稱  磷酸化上皮鈉通道β2抗體
別    名  SCNN1B(phospho S633); 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; Nonvoltage gated sodium channel 1 subunit beta; SCNEB; SCNN 1B; SCNNB_HUMAN; Sodium channel nonvoltage gated 1 beta (Liddle syndrome); Sodium channel nonvoltage gated 1 beta; SCNNB_HUMAN.
濃    度  1mg/1ml