Code
OSR00125W
$240USD
ID Tag
Rb116-281007-WS
Immunogen
A synthetic peptide from aa region 570-640 of human SCNN1B conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
Beta-ENaC, Nonvoltage-gated sodium channel 1 subunit beta, SCNEB, Beta-NaCH, ENaCB, ENaCbeta
Target
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.
SUBCELLULAR LOCATION: Apical cell membrane; Multi-pass membrane protein. Note: Apical membrane of epithelial cells.
DISEASE: Defects in SCNN1B are a cause of autosomal recessive pseudohypoaldosteronism type 1 (PHA1). 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. 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.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Voilley N, et al. Genomics 28:560-565(1995).
2. McDonald F.J, et al. Am. J. Physiol. 268:C1157-C1163(1995).
3. Saxena A, et al. Biochem. Biophys. Res. Commun. 252:208-213(1998).
Limitation
For research use only
Related
products
Code
OSR00125W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB (using recombinant protein). A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for SCNN1B.
Spcs X-react.
Human. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.