Sodium Channel, Nonvoltage-Gated 1, beta (SCNN1B) (AA 570-640) antibody

Details for Product No. ABIN350059
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Antigen
Synonyms besc1, beta-ENaC, enacb, enacbeta, scneb, BESC1, ENaCb, ENaCbeta, SCNEB, BETA-ENAC, SCNN1B, RNENACB
Epitope
AA 570-640
(20), (19), (19), (18), (9), (6), (3), (2), (1), (1), (1), (1), (1), (1)
Reactivity
Human
(83), (78), (63), (35), (35), (34), (34), (1)
Host
Rabbit
(99), (2), (1)
Clonality
Polyclonal
Conjugate
Un-conjugated
(5), (5), (5), (4), (3), (3), (3), (3), (3), (3), (3), (3), (3), (3), (3), (3), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (IHC), Western Blotting (WB)
(55), (42), (32), (20), (19), (19), (11), (11), (3), (2)
Pubmed 5 references available
Quantity 100 µL
Options
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Catalog No. ABIN350059
454.67 $
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Immunogen A synthetic peptide from aa region 570-640 of human SCNN1B conjugated to an immunogenic carrier protein was used as the antigen.
Specificity Specific for SCNN1B.
Purification Whole serum
Alternative Name SCNN1B
Background 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: 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.
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) [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. 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. Also known as: Beta-ENaC, Nonvoltage-gated sodium channel 1 subunit beta, SCNEB, Beta-NaCH, ENaCB, ENaCbeta.
Research Area Cancer, Metabolism
Application Notes 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.
Restrictions For Research Use only
Format Lyophilized
Reconstitution Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.
Handling Advice Avoid freeze and thaw cycles.
Storage 4 °C/-20 °C
Storage Comment 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
General Voilley, Bassilana, Mignon et al.: "Cloning, chromosomal localization, and physical linkage of the beta and gamma subunits (SCNN1B and SCNN1G) of the human epithelial amiloride-sensitive sodium channel." in: Genomics, Vol. 28, Issue 3, pp. 560-5, 1996 (PubMed).

McDonald, Price, Snyder et al.: "Cloning and expression of the beta- and gamma-subunits of the human epithelial sodium channel." in: The American journal of physiology, Vol. 268, Issue 5 Pt 1, pp. C1157-63, 1995 (PubMed).

Shimkets, Warnock, Bositis et al.: "Liddle's syndrome: heritable human hypertension caused by mutations in the beta subunit of the epithelial sodium channel." in: Cell, Vol. 79, Issue 3, pp. 407-14, 1994 (PubMed).

Pirozzi, McConnell, Uveges et al.: "Identification of novel human WW domain-containing proteins by cloning of ligand targets." in: The Journal of biological chemistry, Vol. 272, Issue 23, pp. 14611-6, 1997 (PubMed).

Saxena, Hanukoglu, Strautnieks et al.: "Gene structure of the human amiloride-sensitive epithelial sodium channel beta subunit." in: Biochemical and biophysical research communications, Vol. 252, Issue 1, pp. 208-13, 1998 (PubMed).

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