产品名称
ENaC β Rabbit Polyclonal Antibody
别名
SCNN1B; Amiloride-sensitive sodium channel subunit beta; Beta-NaCH; Epithelial Na(+) channel subunit beta; Beta-ENaC; ENaCB; Nonvoltage-gated sodium channel 1 subunit beta; SCNEB
蛋白名称
Amiloride-sensitive sodium channel subunit beta
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=6338
Human Swissprot No.
P51168
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P51168/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=20277
Mouse Swissprot No.
Q9WU38
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9WU38
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=24767
Rat Swissprot Link
http://www.uniprot.org/uniprot/P37090
免疫原
The antiserum was produced against synthesized peptide derived from human Nonvoltage-gated Sodium Channel 1. AA range:581-630
特异性
ENaC β Polyclonal Antibody detects endogenous levels of ENaC β protein.
稀释度
WB 1:500-2000 IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:20000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
Nonvoltage-gated, amiloride-sensitive, sodium channels control fluid and electrolyte transport across epithelia in many organs. These channels are heteromeric complexes consisting of 3 subunits: alpha, beta, and gamma. This gene encodes the beta subunit, and mutations in this gene have been associated with pseudohypoaldosteronism type 1 (PHA1), and Liddle syndrome. [provided by RefSeq, Apr 2009],
组织表达
Detected in placenta, lung and kidney (PubMed:7762608). Expressed in kidney (at protein level) (PubMed:22207244).
细胞定位
Apical cell membrane ; Multi-pass membrane protein . Cytoplasmic vesicle membrane . Apical membrane of epithelial cells. .
信号通路
Taste transduction;Aldosterone-regulated sodium reabsorption;
功能
disease: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.,disease:Defects in SCNN1B are a cause of Liddle syndrome [MIM:177200]. 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.,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.,PTM:Phosphorylated on serine and threonine residues.,similarity:Belongs to the amiloride-sensitive sodium channel family.,subcellular location:Apical membrane of epithelial cells.,subunit: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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.