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产品信息
OSR00125W-100UL
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
已发表种属
Not Applicable
宿主/亚型
Rabbit
/ Ig
分类
Polyclonal
类型
Antibody
抗原
A synthetic peptide from aa region 570-640 of human SCNN1B conjugated to an immunogenic carrier protein was used as the antigen
偶联物
形式
Lyophilized
浓度
Conc. Not Determined
保存液
whole serum
内含物
no preservative
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. Glycerol (1:1) may be added for added stability.
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.
Specificity of this antibody: SCNN1B.
靶标信息
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. Heterotetramer of two 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. Defects in SCNN1B are a cause of autosomal recessive pseudohypoaldosteronism type 1 (PHA1) [MIM:264350]. PHA1 is a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. There are 2 forms of PHA1: the autosomal recessive form that is severe, and the dominant form which is more milder and due to defects in mineralocorticoid receptor. Autosomal recessive PHA1 is characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatraemia, hyperkalaemia, metabolic acidosis, failure to thrive and weight loss. Defects in SCNN1B are a cause of Liddle syndrome. 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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: Amiloride-sensitive sodium channel subunit beta; amiloride-sensitive sodium channel subunit beta 1; Beta ENaC; Beta-ENaC; Beta-NaCH; Epithelial Na(+) channel subunit beta; epithelial sodium channel beta-2 subunit; epithelial sodium channel beta-3 subunit; nasal epithelial sodium channel beta subunit; Nonvoltage-gated sodium channel 1 subunit beta; SCNEB; SCNN 1B; sodium channel, non voltage gated 1 beta subunit; sodium channel, non-voltage-gated 1, beta subunit; sodium channel, nonvoltage-gated 1, beta
基因别名: BESC1; ENaCb; ENaCbeta; SCNEB; SCNN1B
UniProt ID: (Human) Q96KG2
Entrez Gene ID: (Human) 6338
